Horseshoe lung is a rare congenital anomaly characterized by the presence of unilateral pulmonary hypoplasia and a midline isthmus bridging the right and the left lung. Almost all cases of horseshoe lung are associated with the scimitar syndrome. The scimitar syndrome, sometimes called the congenital pulmonary venolobar syndrome, is a rare but well-described constellation of cardio-pulmonary anomalies. The characteristic abnormality is anomalous pulmonary venous return from a part of or the entire right lung to the inferior vena cava. We present a case of horseshoe lung associated with scimitar syndrome and pleural lipoma in an adult admitted for acute chest pain and chronic dyspnea.