Patients with arteriopathies including giant cell arteritis, Marfan syndrome, and Takayasu's disease are at risk for aneurysmal degeneration of the aorta. Aortic repair has been recommended for these patients to prevent rupture. The purpose of this study was to examine outcomes following endovascular stent graft (EVSG) repair of aortic aneurysms in this patient population. Over an 8-year period, 11 patients (six men, five women) with arteriopathies underwent endovascular aortic repair. The mean age was 50 (range 15-81). Diseases included Marfan syndrome (n = 6), Takayasu's disease (n = 3), and giant cell arteritis (n = 2). Success of EVSG repair was evaluated per the reporting standards of the Society for Vascular Surgery/American Association for Vascular Surgery. Follow-up was a mean of 28.9 months (range 3-68). Six patients underwent EVSG repair of the thoracic aorta, four underwent EVSG repair of the abdominal aorta, and one underwent a staged repair of the thoracic and subsequently the abdominal aorta. Six true aneurysms and six pseudoaneurysms were repaired. Eight patients had previous aortic surgery, including four with multiple aortic operations. For the 12 aneurysms treated, technical success was achieved in 11 (91.7%). One technical failure occurred due to a small iliac access vessel, requiring an eventual iliac conduit for insertion. Early complications (<30 days) occurred in three patients. Type I or III endoleak developed following two repairs (16.7%). Aneurysm expansion occurred following one repair (8.3%). No aneurysm-related deaths occurred during follow-up. EVSG repair of aortic aneurysms is feasible and can be safely performed in patients with arteriopathies. Long-term durability in this younger group of patients who carry an ongoing risk of arterial degeneration remains to be determined.