SummaryWe review the pathophysiology, etiology, clinical manifestations and treatment of the superior vena cava syndrome (SCS). A new etiology of SCS is described: the SCS caused by a central venous catheter which might become more frequent in the next years. The therapy of the neoplastic diseases which are responsible of 97 % of the SCS is discussed, as well as the introduction of chemotherapy as a first line treatment for small-cell anaplastic lung carcinoma.