A 33-year-old woman underwent aortic root replacement for aortic regurgitation and an aneurysm of the ascending aorta due to annuloaortic ectasia. Ehlers-Danlos syndrome was diagnosed by skin biopsy when she was 23 years old. At operation, to avoid mechanical stress to the residual aorta, cardiopulmonary bypass was established via cannulation of the left femoral artery and we used the open distal anastomosis method under hypothermic circulatory arrest with selective cerebral perfusion. Moreover, the sutures of the aortic annulus were reinforced sewing the aortic wall together. Her postoperative course was uneventful. Despite the fragility of the cardiovascular tissues in Ehlers-Danlos syndrome, cardiac surgery could be performed safely with appropriate surgical procedures.