We report a surgical case of severe Ebstein's anomaly associated with pulmonary atresia in the neonate. The baby had remarkable cardiomegaly (CTR > or = 90%) soon after birth and presented severe respiratory distress. He underwent modified Starnes operation (closure of tricuspid valve using a perforated patch, enlargement of interatrial communication, modified Blalock shunt, and PDA ligation) at the age of 12 days. He survived the procedure and cardiopulmonary failure was improved. However, he died from arrhythmia on the 3rd postoperative day. We think this procedure is useful regard to improvement of cardiopulmonary failure due to this fatal congenital heart disease.