Recently the surgical results of simple (isolated) total anomalous pulmonary venous connection have improved markedly, and even the most severely affected patients presenting with marked hypoxia soon after birth have become salvagable partly due to the perioperative use of nitric oxide and extracorporeal membrane oxygenation. However, the outcome after complex surgery, particularly in asplenia, is still very poor in newborns. Common pulmonary vein atresia is still an ominous diagnosis and according to the literature only a few patients have survived surgery. The results of reoperation for postoperative pulmonary vein stenosis are also poor, although the good results of the "sutureless technique" which have recently been reported are encouraging. Prenatal diagnosis using echocardiography is difficult, but postnatal diagnosis using echocardiography and magnetic resonance imaging is usually precise and can obviate the need for cardiac catheterization which often aggravates the hypoxic status in critically ill infants.