Abstract : The superior vena cava (SVC) syndrome associates a series of signs and symptoms caused by an obstruction of the superior vena cava. The etiologies are multiple, but the most common cause is external compression of this vein by a tumor process. The present report concerns a young female with SVC syndrome. The etiology has been shown to be extra-skeletal Ewing’s sarcoma extending from the right atrium to the left brachiocephalic trunk and right humeral vein. This diagnosis of tumor was delayed in the present clinical case and the patient developed serious iatrogenic complications during the early hospital management.