Scimitar syndrome is a rare congenital anomaly consisting a part of right pulmonary venous return to the inferior vena cava. There is a clear bimodal presentation of this syndrome with either infantile presentation or a paediatric/adult form. The infantile variant is severe form with significant mortality. The adult form is less severe and may be asymptomatic. The case we are reporting presented with respiratory distress , hypoplasia of the right lung, dextroposition of the heart and absent left kidney.