Objective: To evaluate the safety and efficacy of emergent endovascular repair for acute Stanford type B aortic dissection with malperfusion syndrome. Methods: Between January 2006 and December 2013, a total of 23 patients with acute Stanford type B aortic dissection and concomitant malperfusion syndrome were admitted, and all patients were reliably diagnosed by CT angiography of the whole aorta before operation. Of the patients, 16 cases were male and 7 were female, and aged 42 to 68 years; 8 cases were complicated with renal malperfusion (6 cases were unilateral), 9 cases had malperfusion of the superior mesenteric artery, 5 cases had unilateral lower extremity malperfusion, and one case suffered from spinal cord malperfusion; 17 cases received emergent endovascular repair and associated adjuvant treatment, and 6 cases who refused the procedure underwent conservative treatment. Results: All the 6 patients undergoing conservative treatment died within 2 weeks. Closure of the primary entry tear with a covered stent-graft was performed in all of the 17 patients who received emergent operation; among whom, malperfusion of the aortic branches was improved in 13 cases after closure of the primary entry tear; in one case with the primary entry tear located at the middle portion of the descending aorta, a bare stent placement was performed first to dilate the true lumen and then the primary entry tear was closed with a covered stent-graft; 3 patients underwent additional bare stent placement for one renal artery or superior mesenteric artery after closure of the primary entry tear. No death occurred in 30 d after operation. During the follow-up period of 3 to 36 months for the 17 patients, one case died due to myocardial infarction 6 months after operation, while all the remaining patients were alive and no stent-related complications were observed. Conclusion: Emergent endovascular repair with restoration of visceral organ blood flow is important method for saving the lives of patients with acute Stanford type B aortic dissection and concomitant malperfusion syndrome.