Cerebral venous thrombosis is a rare disorder with highly variable and nonspecific clinical presentations. For these reasons, specific treatment should be given only when the diagnosis has been firmly established. Etiologic diagnosis should begin in the emergency department to identify underlying conditions that require specific treatment. The mainstay of treatment is anticoagulation with heparin, even in the case of cerebral hemorrhage, followed as soon as possible by oral anticoagulant administration. The optimal duration of oral anticoagulation has not been established. By analogy with systemic venous thrombosis, it should be prolonged 3 to 6 months. When a high risk of recurrence is present, treatment should be continued until the risk disappears. In contrast to arterial stroke, complete recovery of prolonged or severe neurologic deficit is possible, justifying initiation of anticoagulation even when the clinical situation seems desperate. For the same reason, aggressive treatment of intracranial hypertension and seizures or status epilepticus is warranted. Screening for extraneurologic venous thrombosis should be done by means of clinical examination and, if necessary, specific imaging procedures. Local thrombolysis is not yet of proven efficacy and safety. It can be used in patients with clinical worsening related to documented extension of the venous thrombosis despite anticoagulation and in the absence of cerebral hematoma. Surgical treatment is limited to external ventricular drainage and suboccipital craniotomy in the very rare cases of cerebellar vein thrombosis with edematous cerebellar infarct.