A 30-year-old man was admitted to the hospital because of type A aortic dissection associated with annuloaortic ectasia (AAE). A magnetic resonance image (MRI), computed tomography of the aorta demonstrated aortic enlargement with dissection, but revealed no evidence of dissection of the aortic arch. A transesophageal echocardiography demonstrated dissection of the aortic arch. A graft replacement of the total aortic root were performed using hypothermic circulatory arrest with retrograde cerebral perfusion and open distal anastomosis. We reconstructed coronary ostiums with carrel patch, and discontinued the operation without major bleeding. No neurological deficit was found postoperatively.