An autopsy case of Neuro-Behçet's syndrome is reported with review of the previous 13 cases. A 48-year-old woman who had recurrent oral aphthae and genital ulcers beginning at the age of 27 years suddenly developed several neurological symptoms including headache, paraplegia and disturbance of speech. She died of septicemia due to the urinary infection 22 years after the appearance of the first symptoms and one year after the neurological manifestation. Autopsy revealed a marked atrophy of the frontal lobes of the cerebral hemispheres. Microscopic changes in the central nervous system were consisted of edema and lymphocytic cuffing around the vessels, small foci of softening of the white matter and accumulation of fat-laden cells around these foci, and demyelination. These histologic lesions showed a diffuse distribution in the entire brain without a characteristic localisation, but involvement of the basilar nuclei and the pons was most intense in the present case as observed in most of the previous reports. Microscopic examination of other organs revealed severe pyelonephritis and fatty changes of the liver.