Cerebral venous thrombosis (CVT) is less frequent than ischemic stroke or intracerebral haemorrhage. Its incidence is comparable to that of acute bacterial meningitis in adults. Because of the increased use of magnetic resonance imaging (MR) for investigating patients with acute and subacute headaches and new onset seizures, CVT are now being diagnosed with increasing frequency. CVT have a more varied clinical presentation than other stroke types as they rarely present as a stroke syndrome. Their most frequent presentations are isolated headache, intracranial hypertension syndrome, seizures, a focal lobar syndrome and encephalopathy. The confirmation of the diagnosis of CVT relies on the demonstration of thrombi in the cerebral veins and/or sinuses by MR/MR venography or veno CT. The more frequent risk factors for CVT are prothrombotic conditions, either genetic or acquired, oral contraceptives, puerperium and pregnancy, infection and malignancy. The prognosis of CVT is in general favourable, as only around 15 % of the patients remain dependent or die. The main intervention in the acute is anticoagulation with either low molecular weight or unfractionated heparin. In patients in severe condition on admission or who deteriorate despite anticoagulation, local thrombolysis or thrombectomy is an option. Decompressive surgery is life-saving in patients with large venous infarcts or haemorrhage. After the acute phase patients remain anticoagulated for a variable period of time, depending on their inherent thrombotic risk. CVT patients may experience recurrent seizures. Prophylaxis with antiepileptics is recommended after the first seizures, in particular in those with hemispheric lesions. There are several ongoing multicentre registries sand trials which will improve evidence-based management of CVT in the near future.