Scimitar syndrome represents a rare variant of partial anomalous pulmonary venous connection with right lung hypoplasia, dextrocardia, and concomitant airway-vessel abnormalities. Surgical correction is preferred in symptomatic patients or in patients with increased left-to-right shunt. In this report, the first case of scimitar syndrome with dual arterial supply and venous drainage to be treated with thoracoscopic approach is presented.