The midaortic syndrome is an unusual entity seen in children and adolescents. It is characterized by severe narrowing of the abdominal aorta with progressive involvement of the renal and visceral branches. Eleven patients (aged 5 months to 15 years) suspected of having midaortic syndrome were examined preoperatively and postoperatively. All patients had hypertension and were examined with midstream aortography. All aortograms showed a smooth, segmental stenosis of the abdominal aorta and severe bilateral proximal renal artery stenosis. In three patients, percutaneous transluminal angioplasty of the renal artery was attempted, two preoperatively and one for a postoperative stricture. None showed long-term success, presumably due to the progressive nature of the disease. Grafts were surgically placed in ten patients and produced successful results in nine.