BACKGROUND: Isolated acute abdominal aortic dissection (IAAAD) is considered an unusual clinical entity and is traditionally treated by open surgical repair. We report our single-center experience during the last 9 years, evaluating the outcomes after endovascular repair in this patient population. METHODS: All patients with a diagnosis of IAAAD treated in our institute were included in this retrospective review. Between January 2004 and December 2012, 38 patients suffering from an acute aortic dissection were referred regionally for intervention to our department: 24 (63.2%) with a thoracic aortic dissection type B and 14 patients (36.8%, all male; median age, 65 years) with an IAAAD. Demographics, perioperative, procedure-specific, and follow-up data were prospectively aggregated in an electronic database. RESULTS: Patients suffering from IAAAD presented with acute abdominal pain (n = 13) and acute lumbar pain (n = 1). Insufferable pain and enlargement of the aorta were the main indications for treatment. Mean (± standard deviation) maximal abdominal aortic diameter at presentation was 3.5 ± 0.94 cm (range, 2.6-5.1 cm). None of these patients had a concomitant thoracic aortic dissection. All patients were treated by endovascular means, with a primary technical success rate of 100%. Two patients (14%) died perioperatively from cardiac causes. Median hospital stay was 4.5 days (range, 3-16 days). During a mean follow-up period of 37.41 ± 23.56 months (range, 8-82 months; median, 32.5 months), two patients experienced primary treatment failure needing reintervention that led to a successful outcome. The cumulative free-from-reintervention rate was estimated 90.9% at 58 months (standard error, 8.7%). A full aortic remodeling without any events was noted in all other patients during the follow-up period. CONCLUSIONS: Based on our series, IAAAD may represent a not so rare clinical entity as generally thought, suggesting that it may be underdiagnosed. Endovascular treatment of IAAAD appears to be a feasible and efficient therapeutic approach and might be considered as the first-line treatment in all patients with suitable anatomy.