The most common clinical manifestations of fibromuscular dysplasia (FMD) are hypertension due to renal artery involvement and transient ischemic attack or stroke due to carotid or vertebral artery involvement. Patients with renal artery FMD and hypertension should undergo primary angioplasty with the goal of curing the hypertension. If the blood pressure fails to normalize following angioplasty, the physician should institute antihypertensive medications according to the recommendations of the Joint National Committee on the Prevention, Detection, and Treatment of High Blood Pressure VII. In patients with cerebrovascular FMD, antiplatelet agents represent the cornerstone of therapy. Percutaneous angioplasty has emerged as the preferred treatment for symptomatic cerebrovascular FMD.