Concomitant replacement of the ascending aorta and heart transplantation are an infrequent procedure. This procedure was most often performed in patients with Marfan syndrome, however, it has its own technical difficulties. Hereby, we report on combined heart transplantation and aortic root replacement using donor's ascending aorta in a 25-year-old man diagnosed with dilated cardiomyopathy and ascending aorta aneurysm. This procedure was successful and beneficial to patients with aortopathy who are candidates for heart transplantation.