The scimitar syndrome is a rare, complex, variable and congenital malformation of the right lung. These anomalies consist of anomalus pulmonary venous drainage of the right lung to the IVC, hypoplasia of the right lung, hypoplastic right pulmonary artery and anomalies of the right hemidiaphragm. But we recently experienced a case of typical scimitar syndrome except pulmonary venous drainage of the right lung to the IVC. This case shows drainage not to the IVC, but to the left atrium. We present this case with the review of literature.