Behcet's disease was originally described as a triple symptom complex of oral aphthous ulceration, genital ulceration, and hypopyon iritis. It is now known to have a wide systemic manifestations. Among them, the central nervous system involvement should be diagnosed earlier because of it's lethal potential. Recently the authors experienced a case of Behcet's disease with CNS involvement. A 51-year-old female patient was admitted due to deterioration of mentality and generalized ache since 2 years prior to admission. The findings on physical examination were compatible with Behcet's disease, but without cerebrospinal pleocytosis. The manifestations were improved with medications of prednisolone, chlorambucil, colchicines, but relapsed relapsed 2 months later during subsequent tapering of prednisolone and chlorambucil. The patient is now on medication again. A case of Behcet's disease with CNS manifestations is reported with review of literature.