Cogan’s syndrome is a rare inflammatory disease characterized by intraocular inflammation and vestibulo-auditory dysfunction. The exact etiology of Cogan’s syndrome is still unknown, but is currently thought to be an autoimmune disease. Cogan’s syndrome can be accompanied with various conditions including fever, arthritis, skin rash, aortitis, central or peripheral nerve system involvement, lymphadenopathy, splenomegaly, diarrhea. We report a case of Cogan’s syndrome accompanied with meningitis.