Behçet's disease BD is a rare, chronic, systemic vasculitis of unknown origin characterized by recurrences of inflammatory episodes that may affect various organs, including the skin, eyes, joints, nervous system, and blood vessels. The vascular involvement in BD, generally referred to as Angio-Behçet, predominantly involves the venous system, although arterial involvement is less common. The arterial complications include stenosis, occlusion, or aneurysm. Peripheral and pulmonary arteries may be involved, while involvement of the splenic artery is exceptionally rare. This report describes an uncommon presentation of BD manifested by the development of a splenic artery aneurysm in a young woman, revealed by hematemesis.