Fibromuscular dysplasia is a rare vascular disease that is characterized as nonatherosclerotic and noninflammatory in nature. This disease most commonly afflicts the renal and cerebrovascular beds but can rarely affect the upper extremity. We present the case of a 76-year-old woman who complained of a symptom complex, congruent with Raynaud's phenomenon on the right side. The patient had evidence of distal ischemia without the classic angiographic evidence of fibromuscular dysplasia on arteriography. The abnormal arterial section of the right brachial artery was resected and grafted with reversed saphenous vein. She has had no reoccurrence of her symptoms and no stenosis of her graft over a 3-year follow-up period.