 This case report describes a rare presentation of granulomatosis with polyangitis, GPA, which is characterized by mild left and right ventricular enlargement, three small masses in the right ventricle, pulmonary artery hypertension, and spleen lesions. The presence of these findings in conjunction with elevated levels of antineutrophil cytoplasmic antibodies, CNCA and PNCA, confirmed the diagnosis of GPA. This case highlights the importance of considering GPA in the differential diagnosis of patients presenting with cardiac involvement. This article was authored by Majgin Mortazavi and Hamid Nasri.