Middle aortic syndrome typically occurs as severe hypertension in young patients who have weak or absent femoral pulses and an abdominal bruit. It results from a diffuse narrowing of the distal thoracic and abdominal aorta, commonly involving the visceral and renal arteries. The clinical presentation, angiographic assessment, and surgical outcome of 10 patients (mean age: 19.5 years) who underwent one-stage revascularization for middle aortic syndrome were reviewed to determine the effectiveness and durability of one-stage revascularization techniques to relieve these complications. All patients were hypertensive (mean blood pressure: 176 mmHg); six (60%) had severe, poorly controlled hypertension, two of whom had previous failed operations for renovascular hypertension and one who presented with malignant hypertension and acute renal failure. Five patients had disabling myocardial insufficiency, only one of whom had documented coronary artery disease. Four patients had intermittent claudication. Aortography showed variable length high-grade midaortic stenosis, nine had visceral artery involvement, and eight had renal artery involvement. All patients underwent one-stage revascularization by a variety of autogenous and prosthetic techniques. The postoperative recovery was uncomplicated in eight of nine patients and was often associated with dramatic reduction in blood pressure. There was a single death from disruption of the thoracic anastomosis in a patient who had diffuse cystic medial necrosis of the aorta. Arterial biopsy in nine patients indicated evidence for both acquired and congenital origins of the midaortic stenosis. Late follow-up evaluation (mean: 4.1 years) showed normal growth and development, preservation of renal function, and relief of myocardial insufficiency in all patients. Seven patients (77%) are cured of their hypertension, and two (23%) have only mild hypertension. These results indicate that one-stage revascularization of patients with middle aortic syndrome can result in effective and durable relief of these severe life-threatening complications.