Abstract: AIM: To analyze the clinical data and follow-up results of endovascular repair (EVR) for atypical type B aortic dissection. METHODS: Clinical data and treatment results were analyzed in ten consecutive patients with type B atypical aortic dissection treated with EVR from April 2009 to October 2009 and all patients were followed up. RESULTS: Ten patients were studied. All were male with a mean age of (54±11) years and mean body weight of (80±6) kg. All had severe sharp chest or back pain. Among them, nine patients had penetrating aortic ulcer, eight patients had intramural hematoma, and seven patients had both conditions. Three patients had pleural effusion and four patients had contrast leakage demonstrated by CT scan. All patients had a hypertension history of (8±10) years (1-30 years) with average systolic pressure of (189±31) mmHg. Six of the 10 patients had hyperlipidemia, and seven had severe atherosclerosis and arterial calcification diagnosed by CT scan. Technical success was achieved in all ten patients with a mean diameter of stent graft at (35.4±1.9) mm and mean length of stent graft at (150±9) mm. Mean time interval from pain onset to stent graft implantation was (19±16) days (range: 6-60 days). Average ICU stay was (1.0±0.6) days (4 h, 2 days) and average hospital stay after operation was (5.9±1.5) days. One patient received one renal bare metal stent at the time of EVR. One patient had mild paraplegia and was diagnosed as left occipital cerebral infarction by CT scan. All patients were followed-up for a mean period of (8.4±2.1) months (range: 5-11 months). One patient experienced sudden death at home 2 weeks after operation with unknown cause. Cardiac multidetector computed tomography (MDCT) examination 4 days and 3 months after operation showed satisfactory recovery in all other patients. CONCLUSION: EVR is a safe and effective treatment with a high success rate for symptomatic type B atypical aortic dissection with high risk of rupture. Short-term results are satisfactory but mid- and long-term results need further follow-up.