Berry syndrome is a very rare congenital cardiovascular anomaly that consists of a distal aortopulmonary window, aortic origin of the right pulmonary artery, an intact ventricular septum, a patent ductus arteriosus, and an interrupted aortic arch. Two cases of Berry syndrome are presented. A one-stage surgical correction for this complex anomaly was successfully performed, and a 1-year follow-up demonstrated favorable outcomes.