Of the associated cardiac defects with absent pulmonary valve, the combination of tricuspid atresia, ventricular septal defect, and aneurysmal dilatation of the pulmonary arteries is very rare. We report the case of a low-birth-weight girl (2,282 g) with this anomaly, which was prenatally diagnosed. Fontan completion was successfully achieved at 16 months of age, following staged palliative procedures including banding of the main pulmonary trunk and plication of the aneurysmal dilated central pulmonary artery.