Nonarteritic and nonarteriosclerotic atypical thoracoabdominal aortic coarctation (ATAC) is a rare, often inherited malformation. If the abdominal aorta is involved, additional stenoses of visceral arteries may occur, making surgical correction difficult. A literature review was done to accumulate information on the etiology, epidemiology, and surgical therapy of ATAC. Detailed reports on the surgical management of 228 ATAC patients were found in world literature between 1951 and 1996. Most patients were operated on during their first two decades of life. In only about 10% of them could associated diseases (neurofi bromatosis in 7%) be identified, leaving a 90% rate of probably inborn malformations. Main preoperative symptom was renovascular hypertension due to suprarenal or inter renal coarctation, often combined with renal arterial stenoses. Although most of the patients had complex procedures for complete revasculariza tion, perioperative mortality was only 5%. More than 90% of the patients were completely free of symptoms after their operation. Aortic patch angioplasty seems to be burdened with a higher rate of long-term complications than aorto-aortic bypass. Consequent follow-up of all of the young patients is recommended.