Objective To provide data on management and outcomes of patients with acute retrograde aortic dissection (AD) originating from a tear in the descending aorta with extension into the aortic arch or ascending aorta. Methods All patients enrolled in the International Registry of Acute Aortic Dissection from 1996 through 2015 were reviewed. Retrograde AD was defined by primary tear in the descending aorta with proximal extension into the arch or ascending aorta. Primary end-points were in-hospital management strategy and mortality. Results We identified 101 retrograde AD patients (67 men; 63.2±14.0 years). During index hospitalization, medical (MED), open surgical (SURG) and endovascular (ENDO) therapies were undertaken in 44, 33, and 22 patients, respectively. The SURG group presented with larger ascending aorta ( P =.04) and more frequent ascending aortic involvement (81.8% 27/33 vs. 22.7% 15/66, P <.001) compared with the MED and ENDO groups. Early mortality was 9.1% 4/44, 18.2% 6/33, and 13.6% 3/22, for the MED, SURG and ENDO groups ( P =.51). A favorable early mortality was observed in patients with retrograde extension limited to the arch (8.6% 5/58) versus into the ascending aorta (18.6% 8/43, P =.14). Early mortality of patients with retrograde AD with primary tear in the descending aorta (12.9% 13/101) was significantly lower than those with classic type A AD presenting with primary tear in the ascending aorta (20.0% 195/977, P =.001). Conclusions A subset of patients with acute retrograde AD originating from primary tear in the descending aorta might be managed less invasively with acceptable early results, particularly among those with proximal extension limited to the arch.