Behçet disease is a systemic inflammatory perivasculitis characterized by recurrent episodes of oral and genital ulcers associated with skin and ocular lesions. Neurological involvement occurs in 5 to 10% of the cases, and isolated meningeal involvement is very uncommon. We report a 21 years old man with a Behçet disease and a history of acute meningeal syndrome in two previous crises and meningoencephalitis in three others. He reported a history of conjunctivitis and oral and genital ulcers. On examination, he presented oral aphthoid lesions, macular edema, involvement of cranial nerves, pyramidal signs and meningeal irritation. The cell count in cerebrospinal fluid was 1800 cells/mm³, with polymorphonuclear predominance. Magnetic resonance imaging showed lesions in basal ganglia and the brainstem. The patient improved dramatically after intravenous steroid therapy.