A case of complete interruption of aortic arch with aortopulmonary window, patent ductus arteriosus, and aberrantly originated right subclavian artery from proximal descending aorta, in a four year old boy is reported in detail. This is the only reported case in Korea, who has had a successful one-stage total anatomical correction of this combination of defects. Under deep hypothermia and total circulatory arrest, aortic continuity was established using patent ductus arteriosus and anterior wall of pulmonary artery, which was anastomosed obliquely to anteromedial side of ascending aorta. Aortopulmonary window was closed using Impra patch via pulmonary arteriotomy. Then pulmonary arteriotomy was reconstructed primarily except at the junction of right pulmonary artery and main pulmonary artery, where a small piece of pericardium was used to close the defect to prevent kinking and narrowing of right pulmonary artery. Postoperative cardiac catheterization demonstrated a good reconstruction.