Scimitar (hypogenetic lung) syndrome is a rare congenital anomaly from a combined vascular and bronchopulmonary (lung bud) malformation. Scimitar syndrome is characterized by partial anomalous pulmonary venous connection (PAPVC) and predominantly right-sided hypoplastic lung with ipsilateral hypoplastic pulmonary artery. PAPVC refers to a specific group of congenital cardiac anomalies caused by an abnormal return of one or more, but not all, of the pulmonary veins to the right side of the heart instead of draining into the left atrium. In scimitar syndrome, the anomalous pulmonary (scimitar) vein usually drains to the inferior vena cava (IVC), often giving the appearance of a curved scimitar sword on chest radiograph. The scimitar vein can also drain into the right atrium, superior vena cava, azygous vein, or other intermediary venous connections. Coexisting cardiac anomalies, such as dextrocardia, atrial septal defect, pulmonary vein stenosis, coarctation of the aorta, ventricular septal defect, and patent ductus arteriosus are commonly associated with this syndrome.