A congenital diaphragmatic hernia is a life threatening condition. It causes pulmonary hypertension with right to left shunting that contributes to severe hypoxemia in neonates. A particulary poor prognosis is predicted if a congenital diaphragmatic hernia is associated with cardiac deformities and pulmonary hypoplasia. We experienced a case of a 3,500 gm female infant with a congenital diaphragmatic hernia and a single ventricle with left pulmonary atresia. Of prime concern was the maintenance of pulmonary vascular resistance, and this was achieved by altering ventilation, inspired oxygen concentration and blood pH. Also postoperative management of pulmonary hypertension is as important as surgical correction of the congenital diaphragmatic hernia. We performed general anesthesia with O2-fentanyl-isoflurane for correction of diaphragmatic hernia. The fentanyl infusion continued after the operation for blunting of stress responses in the pulmonary circulation. The patient was given a Blalock-Taussig shunt at postoperative day 12.