Cerebral venous thrombosis (CVT) was formerly considered a rare disorder, associated with an unfavorable outcome. More recent data based on modern imaging techniques, however, have changed our perception of this disorder. The use of angiography and, especially, MRI have allowed an early diagnosis and have proved that the incidence of CVT is, in fact, higher than previously thought, approximately 3-4 cases per million people per year, and that the majority of patients have a favorable outcome. At present, the most frequent causes are oral contraceptives assumption and pregnancy/puerperium; as a consequence, 75% of patients are females. CVT may cause isolated intracranial hypertension or lead to an ischemic stroke, which does not follow the distribution of an arterial vessel and has a relevant vasogenic edema. Venous strokes often have a hemorrhagic component, ranging from small petechiae to an actual intracerebral hemorrhage; the latter is associated with a worse clinical course. The clinical presentation of CVT is highly variable and includes patients with just a mild headache, others with focal neurological deficits and a few with a dramatic syndrome with coma; seizures are a frequent presenting symptom. The best radiological examination to confirm the suspicion of CVT is MRI of the brain, which can both demonstrate parenchymal lesions and directly show evidence of sinus occlusions. The available evidence suggests that anticoagulants are effective in reducing mortality and dependency in CVT patients; the possible role of systemic or localized thrombolysis is still to be established.