Behçet's disease is a multisystemic, recurrent, inflammatory disorder, which has a three-symptom complex comprising uveitis, oral aphtae and genital ulcerations. It is rare in childhood. The prevalence of neurologic involvement in BD is range of 10-49%, and shows a wide spectrum from isolated headache to subacute encephalopathy and severe psychosis. We report a 12-year-old Behçet's disease patient with secondary pseudotumor syndrome due to cerebral vein thrombosis and aim to review the literature. © 2006 European Paediatric Neurology Society.