Two infants with asplenia syndrome underwent repair of total anomalous pulmonary venous connection (TAPVC) without cardiopulmonary bypass (CPB). Both patients had other complex cardiac anomalies; pulmonary atresia, univentricular heart, etc. The first patient previously received a left modified Blalock-Taussig shunt. The common chamber (pulmonary venous confluence) was anastomosed to the right side atrium using a side-biting clamp. Though both patients had good postoperative course immediately after surgery, they subsequently died 43 days and 4 months after surgery, respectively. We conclude that relief of TAPVC can be accomplished without CPB and allowing easier and simpler postoperative care.