Behçet's disease (BD) is a chronic, multisystem and relapsing vasculitis of unknown etiology. Central nervous system (CNS) involvement is reported in 30% of cases, but it is the first symptom of the disease in only 5% of subjects. Neurological manifestations may appear as a parenchymal CNS pattern (the commonest), an intracranial hypertension-like pattern, or a meningitis-like pattern. We describe a 30-year-old Algerian man with BD who developed, as first symptom, a typical intracranial hypertension picture with headaches, bilateral papilledema and raised cerebrospinal fluid (CSF) pressure. Magnetic resonance angiography (MRA) revealed a cerebral venous sinus thrombosis (VST). After 1 month of treatment with anticoagulants, prednisone, colchicine and chlorambucil, MRA showed complete recanalization of the cerebral venous sinus and the patient made a full recovery. BD should be routinely looked for in adult patients, especially males in their third and fourth decades, who present with intracranial hypertension syndrome. We therefore advocate the use of MRA for unexplained neurological symptoms in BD, since without it cerebral VST may easily be missed.