 Abstract Fabry disease, FD, is a progressive x-linked inherited disorder caused by deficient or absent lysosomal galactosidase activity. It affects both males and females, with males being more severely affected. The disease causes accumulation of globotryosilceramide within lysosomes, which triggers a cascade of cellular events leading to various symptoms such as pain, angiokeratoma, protenuria, cardiomyopathy, and transient ischemic attacks. Diagnosis is done by enzyme analysis or genetic testing. Treatment options include enzyme replacement therapy using recombinant human galactosidase A and conventional management with pain relief, nephroprotection, and anti-aridmic agents. The long-term outcome of the disease is limited due to progressive damage to vital organ systems, leading to end-stage renal disease and life-threatening cardiovascular or cerebrovascular complications. Long-term enzyme therapy can halt disease progression, but adjunctive therapies should be emphasised, and research is ongoing for an oral therapy. This article was authored by German Dominik P. We are article.tv, links in the description below.