A 20-year-old man with Behçet's disease presented with a ruptured renal artery aneurysm. This patient had previously had aneurysms of the coronary arteries and coronary vein thrombosis that were treated with immunosuppression. A selective transcatheter embolization of the renal artery branch was done successfully and treatment with corticosteroids and methotrexate was added. Presented here is a rare complication of Behçet's disease, with discussion on the pathophysiology, differential diagnosis, and the advantages and disadvantages of the angiographic treatment. This paper is supplemented with a comprehensive review of the literature.