The scimitar syndrome is a rare congenital cardiopulmonary malformation. In association with a horseshoe lung it is extremely uncommon. We describe a child with typical scimitar syndrome (anomalous right pulmonary venous return, hypoplasia of the right lung with dextro-position of the heart and aberrant systemic arterial supply of the right lung), associated with a horseshoe lung. No clinical symptoms are present, surgical management is not necessary. This is the 20th case in addition to the 19 reported cases with scimitar syndrome associated with horseshoe lung. Eight other additional cases of horseshoe lung without scimitar syndrome are mentioned here. In horseshoe lung the pulmonary parenchyma extends from the right lung base across the midline and fuses with the left lung. In only 3 cases continuous parenchymal tissue could be found histological. In 8 cases the lungs were separated by fissurelike structures, seen in CT or chest film, and in 5 cases fissures were demonstrated histological. From 6 patients no findings are available. The various symptoms, the diagnostic tools and the therapeutic procedure will be discussed.