We experienced a case of anomalous origin of right pulmonary artery from the ascending aorta associated with pulmonary atresia, ventricular septal defect, absence of left pulmonary artery afld multiple major aortopulmonary collateral artery (MAPCA). At ten month of age, left pulmonary artery creation with unifocalization and right pulmonary artery banding were performed as the Urst stage, followed by coil embolization of right MAPCA 1 month later, and 1 year later, the total correction was done. After total repair, the patient showed good postoperative course and excellent angiographic and hemodynamic results at 1 year follow-up study.