Almost all cases of isolated levocardia or situs inversus with levocardia are associated with congenital heart disease. We report a case of situs inversus with levocardia, double-outlet right ventricle, atrioventricular discordance, pulmonary atresia, patent ductus arteriosus, atrial septal defect, and ventricular septal defect in a neonate. An overview of anatomy and relevant definitions with radiographic correlation will be presented along with a synopsis of current medical literature.