Total anomalous left pulmonary venous connection with intact atrial septum is an extremely rare form of congenital heart disease. We describe a case never reported before in which the left pulmonary veins drained directly into the right atrium through a common vein. Surgical correction was successfully obtained by redirecting the blood flow through a newly created atrial septal defect into the left atrium using a gluteraldehyde-treated autologous pericardial patch. Follow-up at 6 months shows no signs of any complication.