 Good morning, everyone. Myself Saurabh Das, junior resident in Nildatam Sarkar Medical College and Hospital, Kolkata. Today, I am going to present a paper of BHL syndrome, spectrum of appearances in a single patient. Introduction. BHL syndrome, it is an autosomal dominant hereditary disease. It is caused by germline mutation of BHL gene located in the short arm of chromosome 3. The diagnosis of BHL can be made clinically when the characteristic clinical history and findings are manifested, such as presence of two or more CNS hemangioblastomas. Genetic testing for heterogeneous germline BHL mutation may be also used to confirm the diagnosis of BHL. Imaging plays an important role in the diagnosis as well as surveillance of the patient with BHL. Around 80% of the patient with BHL inherited disorder from an affected parent. The mean age of initial tumor diagnosis of BHL is 26 years, though it ranges from 1 to 70 years, while it may arise de novo in 20% of cases. The clinical diagnosis of BHL can be made out of three of the circumstances. First in a patient with family history of BHL and at least one of the characteristic BHL related tumors such as retinal hemangioblastoma, CNS hemangioblastoma, DSLRCC, antietic nets and endolympatic sac tumors. Second in the presence of two or more retinal or CNS hemangioblastoma or it can be in the presence of one retinal or CNS hemangioblastoma plus at least one of the characteristics of BHL-related visceral tumor excluding the renal and epidermal cyst. These are the manifestations of BHL. For retina it can be retinal hemangioblastoma, CNS, cerebellar and spinal hemangioblastoma, head and neck, endolympatic sac tumor. For pancreas it can be manifest as pancreatic cysts, serocystidinoma or pancreatic neuroendocrine tumor. For kidney it can manifest as renal cyst or clear cell renal carcinoma. For adrenal gland it can represent as pheochromocytoma. For organ it appears as epidermal cyst, papillatic cystidinoma of epidermis or broad ligament cystidinoma. This is the case report. A 33 year old female patient presented with the complaints of diffuse abdominal pain and blaring of vision since six months. On her physical examination revealed that she had a decreased visual activity and a vague abdominal lump in the central abdomen. Following a laboratory investigation that her adrenal function was mildly deranged, in at first the ultrasound of the abdomen revealed that there is a heterogeneous hypolytic lesion as well as cystic lesion both in kidneys and pancreatic panchaema that lead to the suspicious. Further when we did a cross sectional imaging with CCT abdomen and followed by MRI of brain as a spine for spinning of the syndromic nature. These are the radiologic findings that we found in our case. We found cerebellar hemangioblastoma which appeared as a well-defined, solid enhancing nodule without cystic component. Spinal hemangioblastoma came as a well-defined, well-defined avidly enhancing spinal mass. Panchaesis appears simple to inverse cyst without any mural nodule, panchaetic endocrine tumor, solid enhancing lesion in panchaetic panchaema demonstrating an enhancement in arterial phase. Renal cyst, renal cell carcinoma, it appears as heterogeneous enhancing hypervascular renal mass. Pheochromocytomas and extradrenal pyrotechnic glioma appeared as a solid arterial phase as avidly enhancing masses. Retinal hemangioblastoma and broad ligaments, stadenoma, which appeared as an enhanced and enhancing hypotensic lesion in the next summer. These are the immediate features of renal cyst. This is a CCT abdomen that shows up our clinical finding. This is for renal cell carcinoma, a well-defined heterogeneous hypotensic lesion was noted in the lower part of the right kidney. This is a panchaetic cyst that appeared. This is for panchaetic neorendructin tumor, who well-defined hypotensic lesion in panchaetic panchaema demonstrated intense but heterogeneous uptake of contrast in arterial phase and heterogeneous enhancement in renal space, which is likely prevalent in panchaetic nets. Adrenal pheochromocytomas, now this is extradrenal pyrotechnic glioma, heterogeneous enhancing hypotensic lesion in pre-vertibular region. This appears as a papillar cyst adenoma, a broad ligament. These are the CCT features in MRI for renal hemangioblastoma. The arrow represents the clinical finding. This is for cerebellar hemangioblastoma. This is a T1 contrast image. Few well-defined overhead hypotensic lesion was seen in the left cerebral hemisphere. This is for spinal hemangioblastoma MRI finding. Now coming to the part of discussion. VHL disease, it is an autosomal inherited autosomal dominant disorder which is characterized by multi-systemic involvement in the form of retinal hemangioblastoma, hemangioblastomas of the central nervous system, endolympathic sac tumor, renal cell carcinoma, pancreatic cyst, neuroendocrine tumor, pyocromocytomas, extradrenal pyrotechnic glioma, and papillar cyst adenomas of the broad ligament. Our case shows that in a patient with multiple characteristics of VHL-related tumors, one should put detailed work-up to rule out the VHL. Renal involvement in the form of renal cyst and RCC is multi-centric and bilateral in at least 75% of the patients. Pancreatic lesions as seen in VHL include pancreatic cysts, cirrhosis, microcystic adenomas, and endocarsinomas. Out of these pancreatic cysts are the most common. Only 7 to 18% of all the patients of the VHL have hyacomocytomas. It was a detection of the lesion on a CT abdomen, but helped us in further evaluating the patient in an MRI of brain and spinal cord. The MRI further detect the hemangioblastomas of the brain and the spinal cord. The patient was operated for the RCC in the kidney and the neuroendocrine tumor for pancreas and was kept on follow-up. The guidelines for follow-up in a patient aged more than 15 years with VHL is as follows. Anual comprehensive ophthalmological examination, annual evaluation of blood pressure and hearing, annual blood test of plasma, metanepthene and 24-hour urine metanepthene, abdominal ultrasound yearly, abdominal MRI with or without contours every 1 to 2 years, MRI brain yearly, MRI spine every 2 to 3 years. The detection of any of the characteristic lesion of VHL should prompt evolution of addictive any additional lesions among the white gamut of VHL which confirm the syndoming nature. Thank you all for all these are the following references that had to made me this paper.