The patient was an 18-year-old man who had been diagnosed as having a bicuspid aortic valve and dilatation of the ascending aorta six years previously. As he grew up, aneurysmal change of the ascending aorta and hypertension in the upper body gradually progressed. Preoperative evaluation showed annulo-aortic ectasia and the following congenital abnormalities: bicuspid aortic valve, hypoplastic aortic arch, and coarctation of the aorta. Composite graft replacement and extended total aortic arch replacement were carried out.