A rare case of the association of distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and interrupted aortic isthmus in a 40-day-old infant is reported. The infant was suffered from two operations with an interval of nine days. At the first operation a 10mm polytetrafluoroethylene prosthesis was inserted instead of the interrupted aortic isthmus and ductus was ligated via the left posterolateral thoracotomy. But the patient could not be weaned from the respirator because of large amount of left-to-right shunt. So the total correction was subsequently performed after an interval of nine days. At the second operation, tunneling of the right pulmonary artery to the main pulmonary artery through the aortopulmonary septal defect was performed using the Dacron patch via a longitudinal transaortic approach and a separate autologous pericardial patch was applied to the longitudinally incised margins of the anterior wall of the ascending aorta. The second postoperative course was relatively uneventful except some respiratory distress and nutritional problems. Now he is at 6 months of age and thrives well without any symptom. Because the success of the surgical repair of this complex anomalies depends upon the accurate diagnosis and meticulous design of each step of procedure prior to operation these problems are also discussed.