To evaluate the effectiveness of endovascular stent grafting for surgical management of aortic pseudoaneurysm in patients with Behcet's disease (BD).We present a single-institution retrospective cohort of patients with aortic pseudoaneurysm and BD treated with aortic stent grafting. Computed tomography imaging was obtained preoperatively in all patients and once within 2 weeks postoperatively, and then annually. Clinical follow-up and erythrocyte sedimentation rate were used to follow BD activity. Immunosuppressant therapy was instituted prior to endovascular treatment unless a contraindication existed.From 1998 to 2012, 10 patients (eight male, two female; median age, 39) with BD and aortic pseudoaneurysm were treated with endovascular stent grafting at this institution. Ninety percent of these patients received immunosuppressive therapy before and after surgical treatment. The median follow-up period was 57 months (interquartile range, 43-72). The locations of the 12 pseudoaneurysms treated in this cohort were infrarenal abdominal aorta (seven), descending thoracic aorta (four), and aortic arch (one). Median pseudoaneurysm size was 4.5 cm (interquartile range, 3.4-5.9). At long-term follow-up, complete resolution of the aortic pseudoaneurysm was noted in all patients. No endoleaks occurred. Newly developed pseudoaneurysm at the distal margin of the stent graft was noted in one patient 17 months after the stent graft procedure. One patient required a subsequent stent graft placement for an expanding pseudoaneurysm of the subclavian artery. No patient deaths occurred during the follow-up period.Endovascular treatment of aortic pseudoaneurysm with stent-grafting in patients with BD is safe and effective with long-term durability.