Cerebral venous thrombosis (CVT) in children is a multifactorial serious disease. It is being increasingly diagnosed, mainly because of more sensitive diagnostic procedures and increasing clinical awareness. The clinical manifestations can be life-threatening and cause long-term neurological deficits. Thromboembolism in children is a multifactorial disorder in which both genetic and acquired risk factors play a role. CVT occurs in various clinical settings, including infection, dehydration, renal failure, trauma, cancer and haematological disorder with multiple risk factors. Clinical manifestations of CSVT are nonspecific and may be subtle. Most of the clinical scenarios occur at all ages and the clinician should consider this diagnosis in a wide range of acute neurological presentations in childhood. CVT can have an extremely variable clinical presentation, mode of onset, imaging appearance and outcome. Its prognosis remains largely unpredictable. Diffusion and perfusion MRI may play a role in detecting venous congestion and CT or MR venography are now the methods of choice for investigation of cerebral venous thrombosis. The options for treatment of infants and children include standard or low molecular weight heparin for 7-10 days followed by oral anticoagulants for 3-6 months. Specific treatment with anticoagulation is controversial in children, but has been established as appropriate therapy in adults. Anticoagulant treatment with heparin is probably safe and beneficial for children with sinus thrombosis, even those with intracranial haemorrhages.