The objective of the present study is to clarify safety and efficacy of thoracic endovascular aortic repair (TEVAR), excluding the primary entry in the descending aorta, for type-B0,D acute aortic dissection (TB0,DAAD) (so-called retrograde type-A acute aortic dissection). Forty-six patients with hyperacute-phase (within 2 days after the onset) type-A acute aortic dissection (TAAAD) and TB0,DAAD underwent urgent (on the admission or next day) intervention (TEVAR or conventional surgical aortic repair [CSAR]) for 2 years. Results of TEVAR for TB0,DAAD were compared with those of CSAR for TAAAD. Outcomes included 30-day mortality, aortic re-intervention, and major complications (stroke and paraplegia/paraparesis). Details of TEVAR were also analyzed. Seven patients with TB0,DAAD and 39 patients with TAAAD underwent respectively urgent TEVAR and CSAR. Aortic re-intervention was significantly more frequent in the TEVAR than CSAR group (28.6% versus 0%, p <0.01). There was no difference in incidence of death and stroke between the TEVAR and CSAR group. All the 7 patients survived and 5 of the 7 (71.4%) patients were relieved of aortic re-intervention for 30 days following TEVAR. One patient, however, underwent aortic arch replacement on postoperative day (POD)1 owing to the patent and non-shrinking ascending false lumen (FL). The entry existed in the ascending aorta. Another patient underwent ascending and transverse aortic replacement with frozen elephant trunk on POD13 due to proximal stent-graft induced new entry (SINE) irrespective of the thrombosed and shrinking ascending FL. Because of the patent and non-shrinking ascending FL, one patient underwent additional TEVAR for the residual entry in the distal descending thoracic aorta on POD33 and subsequently ascending aortic replacement 4 months later. No entry was detected in the ascending or transverse aorta. The ascending FL in the other 4 patients was thrombosed early, shrinking gradually, and disappeared at last following TEVAR. Urgent TEVAR for TB0,DAAD may be alternative to CSAR in selected cases. Accurate diagnosis of the primary-entry location on pre-interventional computed-tomography scans for exclusion of the entry and cautious selection and delivery of a stent graft to prevent SINE or endoleak are requisite for success of the procedure, remodeling of the FL, and satisfactory prognosis.