A 21-year-old male patient had suffered from palpitation and exertional dyspnea since October, 1997. He was admitted to our hospital, and a series of examinations were performed. Chest computed tomography (CT) revealed marked dilatation of the ascending aorta (about 7.5 cm at the proximal portion) and aortic annulus, an intimal flap in the ascending aorta and aortic arch was also noted. Cardiac catheterization revealed the pulmonary capillary wedge pressure was 33 mmHg, pulmonary artery pressure was 47/38 mmHg with a mean of 35.4. The cardiac index was 1.01 l/min/m2. Poor left ventricular contractility was shown by a left ventricular ejection fraction (LVEF) of 13.8% and a right ventricular ejection fraction (RVEF) of 5.13% by a radionuclide angiogram (RNA) study. Under the diagnosis of dilated cardiomyopathy and dissecting aortic aneurysm of the ascending aorta and aortic arch, he was put on a waiting list for heart transplantation. On November 11, 1997 he received heart transplantation. Resection of the dissecting aneurysm of the ascending aorta and the aortic arch and replacement with a 26 mm Vascutek graft were performed first under deep hypothermia and retrograde cerebral perfusion. Then while he was rewarming up, heart implantation was performed. He was discharged 30 days after surgery and has been doing well since then. As far as we know, no literature regarding combined heart transplantation and resection of a dissecting aneurysm of the ascending aorta and aortic arch has been reported.