The right ventricle to pulmonary artery connection with an extracardiac conduit, left pulmonary artery reconstruction, ligation of patent ductus arteriosus, and take-down of right Blalock--Taussig shunt were performed on a 1-year-8-month-old boy who had pulmonary atresia, ventricular septal defect, patent ductus arteriosus, and major aortopulmonary collateral arteries. He previously underwent the unifocalization and right modified Blalock--Taussig shunt at 9 months of age. He repeatedly had a difficulty in weaning from the mechanical ventilator. After removing the aneurysm from the divided major aortopulmonary collateral artery that compressed the left main bronchus externally, it was possible to wean him from the mechanical ventilator.