A 74-year-old woman was admitted to the hospital with sudden onset of a blue, painful right index finger, and the condition progressed to digital ulceration. The ulcer gradually healed over a period of 2 months. Peripheral pulses, Doppler-derived brachial and radial systolic pressures, and results of an echocardiogram were normal. Digital pulse volume recordings showed obstructive waveforms in all digits of the right hand. Arteriography showed a normal arch and normal innominate, subclavian, and axillary arteries. The midbrachial artery was markedly abnormal and had alternating areas of stenosis and aneurysm formation. Multiple occlusions involved the palmar arch and proper digital arteries. The abnormal brachial artery segment was excised and replaced with an autogenous reversed saphenous-vein conduit. Histologic examination confirmed the lesion to be medial fibromuscular dysplasia. Fibromuscular dysplasia that involves upper extremity arteries is extremely uncommon and rarely presents with digital artery embolization. This case emphasizes the importance of exclusion of proximal reconstructible arterial occlusive disease by means of complete arteriographic examination of patients who were admitted with unilateral finger or hand ischemia.