Horseshoe lung is a rare congenital malformation in which the pulmonary parenchyma connects in the midline posterior to the pericardium and anterior to the aorta and esophagus. There have been 51 case reports in the world literature. About 80% of these have been associated with Scimitar syndrome, and most cases are diagnosed in infancy due to pulmonary symptoms. To our knowledge, this is the first case report of horseshoe lung presenting in an adult and associated with a pulmonary sling, complete bowel malrotation, and renal ectopia.