A 5 year-old girl with pulmonary atresia with intact ventricular septum underwent a modified Fontan operation successfully. In spite of the previous Brock and shunt procedures, the right ventricle remained hypoplastic as end diastolic volume of 50% of normal with hypoplastic tricuspid valve. A modified Fontan operation was performed as a definitive operation with main reason of hypoplastic tricuspid valve. The selection of the definitive procedures was discussed.