Behçet disease is a rare multisystem immune-related vasculitis that is prevalent in Japan, the Middle East, and many Mediterranean countries. In our study population of 36 patients with Behçet disease, 16 (44%) had CNS involvement. CT was noncontributory, except in a single patient with dural sinus thrombosis. MR imaging in nine patients showed foci of high signal intensity on T2-weighted images within the brainstem, the basal ganglia, and the cerebral hemispheres in that order of frequency. Unlike the plaques associated with multiple sclerosis, Behçet lesions show no predilection for the periventricular regions.