A 10-day-old neonate with Darling type Ia of total anomalous pulmonary venous connection is presented. The left pulmonary vein entered the right thoracic cavity behind the right atrium and drained into the innominate vein together with the right lower, middle and upper pulmonary veins. The patient was treated with right-sided approach. The postoperative course was uneventful. To our knowledge, this case is the first successful correction of this subtype of anomaly in Japan.