Scimitar syndrome a very rare and variable congenital disorder characterized by an anomalous connection of the pulmonary vein with the IVC. The syndrome is mostly seen in very early infancy, but was now recognized in a 46-year-old woman, who was referred to the outpatient clinic of the department of cardiology with complaints of dizziness. Contrast enhanced computer tomography (CT) showed dextroposition of the heart and a large right pulmonary vein joined the inferior vena cava (IVC) just above the level of the diaphragm. The typical features of the syndrome are discussed.