A Rastelli procedure was successfully performed on a 6-year-old girl with an absence of the intrapericardial pulmonary artery and the left pulmonary artery (PA), following a right B-T shunt. The central PA was reconstructed with an 18 mm diameter xenopericardial roll behind the ascending aorta and the superior vena cava. After intracardial repair, the Rastelli operation was performed using an 18 mm diameter composite graft which consisted of a valved xenopericardial roll and a knitted Dacron graft. Since the size of the right PA was large enough to undergo this surgical procedure (the preoperative right PA index was 300 mm2/m2), the postoperative peak systolic pressure ratio of the right ventricle to the left ventricle declined from 1.0 to 0.64. Although the patient showed slight signs of right ventricular failure on the operative day, the postoperative course was uneventful.