This reports a successfully corrected case of an 8-day-old baby who had a rare mixed type of total anomalous pulmonary venous connection in which the left pulmonary vein connected to the portal vein and the right one was atretic as demonstrated by pulmonary artery wedge angiography. The left common pulmonary vein and right pulmonary veins were anastomosed to the left atrium, separately. The patient tolerated the operation and has been well. Early total repair after accurate diagnosis was successful for this rare combination of anomalous pulmonary venous connection.