Superior vena cava (SVC) syndrome refers to the clinical manifestations of cerebral venous hypertension secondary to obstruction of the SVC and/or the innominate veins. The most common cause of SVC syndrome is malignancy like small cell lung cancer and non-Hodgkin lymphoma, but there is an increasing trend of benign etiologies secondary to thrombosis due to central lines/ pacemakers or mediastinal fibrosis. Supportive measures include head elevation, diuresis, supplemental oxygen, and steroids. Thrombolysis with or without endovenous stenting is required emergently in those with airway compromise or symptoms secondary to cerebral edema. Definitive treatment in those with malignancy is multidisciplinary; this requires radiotherapy, chemotherapy, SVC stenting, oncologic surgery and SVC bypass or reconstruction. Endovascular treatment is the primary modality for palliation in malignancy and in those with benign etiology. Surgery is reserved for those who have failed or are unsuitable for endovascular treatment. In patients with benign disease endovenous stenting and open surgery provide excellent symptom relief and are safe and effective.