Cerebral venous and sinus thrombosis is a still underdiagnosed cause of stroke, with an incidence of about 2.8 events per 100,000 person-years in young women and about 1.3 events per 100,000 person-years in the general population. Puerperium, oral hormonal contraception, and coagulation disorders remain the most frequently identified risk factors. Initial treatment with heparin is the only proven therapy, although the evidence is based on only two randomized placebo-controlled trials which together included 79 patients. In the case of clinical deterioration under anticoagulation, local thrombolysis and mechanical thrombectomy may be considered, but clinical efficacy is supported only by case reports. Patients with imminent lateral herniation due to large hemorrhagic infarctions should be treated with prompt surgical decompression. Following the acute phase, oral anticoagulation is recommended for 3-12 months, and only patients suffering from a severe coagulopathy or with recurrent cerebral venous and sinus thrombosis should be considered for long-term anticoagulation. Only insufficient experience is available for novel anticoagulants such as thrombin inhibitors or factor Xa antagonists.