Cerebral venous and sinus thrombosis (CVT) is a rare form of thrombosis, with many different clinical manifestations. Better imaging techniques have greatly improved the diagnosis, but as a result of the paucity of controlled trials, choosing the optimal treatment for each patient often remains a challenge. Heparin is generally considered the mainstay of treatment, supported by data from a few small trials. More invasive treatment options are available, such as endovascular thrombolysis and--in more severe cases--decompressive hemicraniectomy. Furthermore, CVT is often accompanied by various neurological complications, such as seizures and intracranial hypertension, which require specific treatment. In this review we summarize the available treatment options for CVT and suggest which therapy should be reserved for which patients.