Behçet disease is a recurrent systemic vasculitis of unknown etiology, that involves vessels of nearly all sizes and types. Because of this, disease manifestations can occur at many sites throughout the body. Central nervous system (CNS) involvement may be parenchymal or nonparenchymal and has a global prevalence that ranges from 3% to 10%. Main signs of CNS involvement are pyramidal and those resulting from brain stem lesions. Aseptic meningitis, mental changes, sphincter disturbances, pseudobulbar syndrome, and deep sensory abnormalities may be seen. Analysis of cerebrospinal fluid, computed tomography (CT), magnetic resonance imaging (MRI), single-photon emission computed tomography (SPECT) and brain angiography offer assistance in the diagnosis. The course of disease can be primary progressive, secondary progressive or have a relapsing-remitting profile. Boluses of methylprednisolone for three days followed by cyclophosphamide are the treatment of choice. This papers discusses these aspects of neuro-Behcet on the basis of complex clinical case