Since the recent report, airway pressure release ventilation (APRV) may improve the hemodynamics than conventional positive pressure ventilation after bidirectional cavopulmonary shunt (BCPS) and Fontan operation. We present the case a four-month-old boy with asplenia syndrome and total anomalous pulmonary venous connection (TAPVC). He had undergone TAPVC repair three days after birth and BCPS three months after birth. Because his superior vena cava (SVC) pressure was 19 mmHg and venous collaterals increased, we could not wean the patient from mechanical ventilation for more than a month. Subsequently, APRV with spontaneous breathing was initiated (F_IO₂ 0.8, T high 2.5 sec, T low 0.5 sec, P high 15 cmH₂O, P low 0 cmH₂O) with the goal of enhancing pulmonary blood flow that is accompanied by a decrease in intrapleural pressure to < 0 cmH₂O; this led to a prompt decrease in SVC pressure (19 mmHg to 14 mmHg) and amelioration of cyanosis. He was extubated four days after APRV initiation (F_IO₂ 0.5, T high 2.2 sec, T low 0.3 sec, P high 6 cmH₂O, P low 0 cmH₂O). This case report indicates that APRV can be one of the options after BCPS in infants.