Total anomalous pulmonary venous connection (TAPVC) with congenital diaphragmatic hernia (CDH) is a disease entity with high mortality rate. Association with atretic left pulmonary artery increased the complexity of the anomalies. Here, we reported a newborn baby with these complex congenital anomalies successfully treated surgically. Over 13 years after surgery, there was substantial growth of left pulmonary artery which was angiographically atretic at his newborn stage, which was rarely reported. Currently, this patient is drug free and is in functional class I of New York heart association.