Cogan’s syndrome is a rare chronic inflammatory disease Characterized by non-syphilitic keratitis and vestibuloauditory dysfunction. Although the precise pathogenesis of Cogan’s syndrome is unknown, it is thought to develop from vasculitis involving multiple organ system. It can be accompanied with various systemic diseases including arthritis, lymphadenopathy, splenomegaly, and aortitis with insufficiency. We report a case of typical Cogan’s syndrome with multiple cerebral infarctions.