Segmental arterial mediolysis (SAM) is a rare condition caused by loss of muscular elements in the walls of medium-sized, usually visceral, arteries. This causes dissection, occlusion, aneurysm formation, and rupture. The clinical presentation is usually catastrophic as a result of vascular occlusion or rupture. Herein an unusual case of renovascular hypertension resulting from SAM is reported, which was successfully treated with balloon angioplasty.