Background Ehlers-Danlos syndrome (EDS) is a disorder in the metabolism of fibrillary collagen. Its vascular type (vEDS) frequently develops arterial rupture and aortic dissection. Case presentation The patient was a 54-year-old gentleman who suffered from cardiac tamponade, malperfusion of the left carotid artery and the right lower extremity due to acute type A aortic dissection. Rupture of the aortic root and a huge entry located from the transverse arch to the proximal descending aorta were found. There were no significant deformities at aortic valve, so we tried extended repairs of valve sparing root replacement (VSRR) and total arch replacement (TAR) with frozen elephant trunk (FET) and were successfully performed. He was finally diagnosed as vascular Ehlers-Danlos syndrome (vEDS) by genetic examination. Conclusion Emergency extensive repairs of VSRR and TAR with FET for critical AAAD with rupture and malperfusion was successfully performed for a vEDS patient.