A neonate with cyanosis at birth was found to have a rare type of totally anomalous pulmonary venous connection. The pulmonary veins entered a confluence posterior to the left atrial wall, which drained into the right superior cavoatrial junction. There were no other major structural defects and no evidence of isomerism. Because of the severe cyanosis, and the restrictive nature of the interatrial communication, we performed balloon atrioseptostomy to improve oxygenation. We then achieved successful surgical repair when the baby was aged 7 months.