Fibromuscular dysplasia is an increasingly recognized cause of vascular disease in young or middle-aged persons and may be amenable to medical or surgical management. We report five patients, one of whom died, with emphasis on the beaded and irregular pattern of change in the carotid arteries. Neck pain, bruit, and transient ischemic attacks are the most usual clinical symptoms. The cause of fibromuscular dysplasia remains obscure, but in view of the increased incidence in young women, hormonal factors may be involved. Dilation or resection can be considered, as in two patients reported here, and anticoagulation may benefit other patients.