The scimitar syndrome, a rare complex anomaly, is defined as an anomalous right pulmonary venous drainage, partial or complete, to the inferior vena cava. The shape of the Turkish curved sword (scimitar) has provided the name of this syndrome. Additional characteristics of this syndrome such as hypoplasia of the right lung and of the right pulmonary arterial tree, anomalous arterial supply of the right lung from the aorta, dextrocardia and bronchial anomalies are common. Recently we experienced a case of scimitar syndrome (adult form) in a 19-year-old woman patient, so we report the case with a brief review of the literature.