Pulmonary Atresia with VSD (PA-VSD) is a complex cyanotic cardiac anomaly previously known as Truncus type IV or Pseudotruncus. For all practical purposes it is same as TOF with pulmonary atresia. Presences of systemic pulmonary collaterals are important association, which sometimes by increasing pulmonary flow may produce CHF. Some neonates born with cyanosis slowly become irritable, hypoxic and critically ill indicating closure of the ductus with inadequate collaterals. Whereas other neonates or infants born with cyanosis gradually become stable because of early development of collaterals and they have retarded growth. Infants present with cyanosis, single S2 (only A2), aortic ejection click and wide spread continuous murmur over precordium due to collateral flow. There is no ejection murmur over pulmonary area. ECG shows right axis deviation with right ventricular hypertrophy, X-ray is like that of TOF but pulmonary vascularity may be uneven due to collaterals. Echocardiography confirms the diagnosis by delineating atretic pulmonary valve along with features of TOF. Major aorto-pulmonary collaterals can also be demonstrated. Right aortic arch may be present. Medical management is mainly by prostaglandin E1 infusion when the neonate is duct dependant but surgical repair is the definitive treatment.