Horseshoe lung (HL) is a rare bronchovascular anomaly in which the posterobasal segments of the right and left lung are fused with a narrow isthmus of pulmonary parenchyma in a common pleural space behind the heart and anterior to the aorta and oesophagus. HL is mostly associated with unilateral lung hypoplasia (most frequently the right lung) in conjunction with the Scimitar syndrome and is also associated with cardiovascular anomalies, such as atrial septal defects, ventricular septal defects, tetralogy of Fallot, and hypoplastic left ventricule. 1 Oguz B. Alan S. Ozcelik U. et al. Horseshoe lung associated with left-lung hypoplasia, left pulmonary artery sling and bilateral agenesis of upper lobe bronchi. Pediatr Radiol. 2009; 39: 1002-1005 Crossref PubMed Scopus (15) Google Scholar , 2 Salerno T. Guccione P. Malena S. et al. Horseshoe lung associated with unique left pulmonary vein: an unreported association. Pediatr Cardiol. 2010; 31: 905-907 Crossref PubMed Scopus (8) Google Scholar , 3 Jeewa A. Culham J.A.G. Human D.G. A case of horseshoe lung and complex congenital heart disease in a term newborn. Pediatr Radiol. 2010; 40: 206-209 Crossref PubMed Scopus (5) Google Scholar HL with left lung hypoplasia is extremely rare, with only a few cases reported in the literature. 1 Oguz B. Alan S. Ozcelik U. et al. Horseshoe lung associated with left-lung hypoplasia, left pulmonary artery sling and bilateral agenesis of upper lobe bronchi. Pediatr Radiol. 2009; 39: 1002-1005 Crossref PubMed Scopus (15) Google Scholar , 2 Salerno T. Guccione P. Malena S. et al. Horseshoe lung associated with unique left pulmonary vein: an unreported association. Pediatr Cardiol. 2010; 31: 905-907 Crossref PubMed Scopus (8) Google Scholar , 3 Jeewa A. Culham J.A.G. Human D.G. A case of horseshoe lung and complex congenital heart disease in a term newborn. Pediatr Radiol. 2010; 40: 206-209 Crossref PubMed Scopus (5) Google Scholar , 4 Ersoz A. Soncul H. Gokgoz L. et al. Horseshoe lung with left lung hypoplasia. Thorax. 1992; 47: 205-206 Crossref PubMed Scopus (23) Google Scholar To the authors’ knowledge, this is the first case of HL associated with left lung hypoplasia and a single left pulmonary vein as depicted using multidetector computed tomography (MDCT) in the literature. These complex bronchovascular anomalies require angiography, bronchoscopy, echocardiography, and also CT for correct diagnosis. With development of MDCT, a non-invasive imaging technique, it is possible to visualize vascular, bronchial, and parenchymal structures during a single imaging session via multiplanar two (2D) and three-dimensional (3D) images. 1 Oguz B. Alan S. Ozcelik U. et al. Horseshoe lung associated with left-lung hypoplasia, left pulmonary artery sling and bilateral agenesis of upper lobe bronchi. Pediatr Radiol. 2009; 39: 1002-1005 Crossref PubMed Scopus (15) Google Scholar , 5 Akay H.O. Kervancıoglu M. Nazaroglu H. et al. Horseshoe lung associated with rare bilateral variant of scimitar syndrome: demonstration by 64-slice MDCT angiography. Pediatr Radiol. 2008; 38: 563-566 Crossref PubMed Scopus (12) Google Scholar