Cerebral venous thrombosis (CVT) is an uncommon cause of stroke that mainly affects young adults and children. In contrast to venous thromboembolism, women are affected three times more often than men. Baseline symptoms can vary considerably between patients, but most present with headache, seizures, or focal neurological deficits. Patients can be diagnosed with magnetic resonance imaging, computerized tomography-venography, or catheter angiography, although the latter is rarely required anymore. Approximately 30-50% of patients have an intracerebral hemorrhage, which can range from a small juxtacortical hemorrhage to large space-occupying lesions. Based on limited evidence from clinical trials, the primary therapy for CVT is anticoagulation with heparin. Uncontrolled studies have shown promising results for the use of endovascular treatment in severely affected patients, but these studies require confirmation in prospective clinical trials. In patients who develop clinical and radiological signs of impending herniation decompressive surgery can be both life saving and result in a good functional outcome.