Pulmonary atresia and ventricular septal defect (PA-VSD) with major aorto-pulmonary collaterals (MAPCAs) is a complex and extremely heterogeneous anomaly. Most untreated patients die in their first decade of life because of intractable congestive heart failure or respiratory distress. PA-VSD is characterized by a wide variety of anatomy of central pulmonary artery and nature of collateral lung perfusion. In most patients, collateral perfusion is provided either by MAPCAs or by patent ductus arteriosus (PDA). The management of infants and children with pulmonary atresia, ventricular septal defect, and MAPCAs has proven to be challenging. Therapeutic approaches include onestage surgical repair, staged unifocalization, shunting, and coiling of collateral vessels. Results have been variable and frustrating. In this case report, we discuss the characteristic, variants, and how far we can manage the patient who suffered from PA-VSD.