The congenital anomaly of aortopulmonary window is rare. Its combination with interruption of the aortic arch has been previously recognized and reported in infants and children. Early and complete repair of such a congenital anomaly may prevent progression to cardiac failure and premature death. We describe the successful echocardiographic diagnosis and one-stage surgical repair, via a median sternotomy, of the aortopulmonary window, interrupted aortic arch, patent ductus arteriosus, and patent foramen ovale in a 3-day-old neonate. The literature is also reviewed.