Common pulmonary vein atresia is a rare congenital anomaly; all four pulmonary veins drain into a common dilated chamber with no direct connections to the heart or systemic venous system. Since its first description in 1962, 16 cases have been reported. Only four patients were surgically managed and none survived. This communication presents the seventeenth reported case of common pulmonary vein atresia and the only patient whose anomaly was suspected early enough to demand immediate surgical management, with gratifying long-term success. The literature on the subject is reviewed and common features of the anomaly are emphasized to facilitate precise diagnosis, so that a futile search for a nonexistent communicating vein is avoided at the time of operation. This approach has led to the first successful surgical management of this otherwise fatal lesion.