Horseshoe lung is a very rare congenital malformation in which the bases of the right and left lung are fused to each other by a narrow isthmus. Although rare, a hyperlucent area in the lower left lung, close to the vertebral column, may represent a horseshoe lung. Horseshoe lung is often associated with scimitar syndrome. Here, we present a case of a 2-year-old girl who presented with recurrent chest infections and wheezing associated with horseshoe lung. The right lung appeared to be hypoplastic.