From June 1974 to December 2001 we performed operative treatment on 33 patients with descending thoracic or thoracoabdominal aortic aneurysm in association with Takayasu disease. There were 25 men and 8 women with a mean age of 40.2A years (range 16-64A years). Nineteen patients came from North Africa, 6 were from France, and 8 were from various locations in the world. The revealing symptom was hypertension in 12 cases, thoracic or abdominal pain in 7, isolated inflammatory syndrome in 5, neurologic or ocular manifestations in 3, rupture in 3, and embolization to the lower extremity in 1. In the remaining two cases discovery was coincidental. The aneurysm was confined to the thoracic aorta in 10 cases and involved both the thoracic and abdominal aorta in 23 cases. There were 8 type I, 6 type II, 4 type III, and 5 type IV aneurysms according to Crawford's classification. Two patients had undergone previous repair of the thoracoabdominal aorta. Four patients required first-stage treatment of a renal artery lesion to control hypertension. Six patients had associated aneurysms of the proximal aorta, including five treated via the distal elephant trunk technique in first-stage procedures. Aneurysm repair consisted of prosthetic replacement of the thoracoabdominal aorta in 31 cases, exclusion bypass in 1 case, and stent graft placement in 1 case. The procedure was performed with cross-clamping alone in 13 cases, distal perfusion in 17 cases, and deep hypothermic circulatory arrest in 3 cases. Twenty patients (61%) had associated renal and/or intestinal artery lesions that were treated during the same procedure as that for the thoracoabdominal aorta in 19 patients (58%). A total of 24 procedures were performed on renal arteries (17 revascularizations, 7 nephrectomies). Associated supraaortic trunks lesions were present in 15 patients (45%) and were treated in 12 patients, including 8 in first-stage procedures prior to thoracoabdominal aortic aneurysm repair. Three patients died of multiple organ failure, after reoperation in two cases and infection in one case involving prior long-term corticosteroid therapy. Three patients developed paraplegia, including one who had undergone emergency treatment following rupture. Two patients required reoperation, for hematoma in one case and bowel necrosis in one. Four patients developed respiratory complications requiring artificial ventilation for more than 48 hr. During follow-up, two patients died from complications after repair of the proximal aorta and one patient required nephrectomy. Despite the extent of aneurysmal lesions and high frequency of association with visceral and supraaortic vessel lesions, the outcome of surgery in patients presenting with descending thoracic or thoracoabdominal aortic aneurysm in association with Takayasu disease was satisfactory.