Moyamoya ("puff of smoke") syndrome is a disease of children and young adults caused by fibromuscular dysplasia of the internal carotid and proximal cerebral arteries and is rarely associated with hypertension. We report a 34-year old woman with moyamoya syndrome who presented with symptomatic severe hypertension associated with unilateral renal artery stenosis. The patient underwent percutaneous transluminal angioplasty of the right renal artery with subsequent improvement in blood pressure control on reduced, and later, no antihypertensive therapy. This case illustrates that moyamoya syndrome may be an intracranial manifestation of a systemic arterial disorder. Renal artery stenosis may, in appropriate cases, be managed by percutaneous transluminal angioplasty.