We present seven patients with the scimitar syndrome. The clinical and anatomical spectrum is described. Two different types of scimitar vein was recognized. a) simple classical vein running from the middle of the right lung to the cardiophrenic angle (5 patients) and b) double arched vein in the upper and lower lung zone, with ample drainage into the left atrium and inferior caval vein (2 patients). Four patients required surgical treatment. Indications, diagnostic procedures and surgical management are discussed. In two patients, thrombosis and fibrosis occurred in the scimitar vein that had been reimplanted in the left atrium, necessitating pneumonectomy.