Cerebral venous thrombosis is an infrequent condition characterized by extreme variability in its clinical presentation and mode of onset. The combination of magnetic resonance imaging and magnetic resonance angiography is currently the best method for diagnosis. The proportion of cases of unknown etiology remains high. The prognosis, although better than previously thought, remains unpredictable. Treatment, which should be started as soon as the diagnosis is established, consists of reversing the underlying cause when known, control of seizures and intracranial hypertension, and the use of antithrombotics. Heparin should be the first-line antithrombotic agent. Recent studies have confirmed its safety even in patients with hemorrhagic parenchymal lesions. Local thrombolysis is indicated in the very rare cases that deteriorate despite adequate anticoagulation. Cerebrospinal fluid diversion or optic nerve fenestration is used for vision-threatening papilledema when intracranial pressure control is difficult.