We report the case of an 18-year-old black woman with urticarial vasculitis, vestibuloauditory dysfunction and superficial keratitis. Oral steroid therapy was effective in reducing most clinical manifestations with high-dosage dependency. However, only intravenous pulses of methylprednisolone allowed slight improvement of hearing. In this case, we emphasize urticarial vasculitis as a new manifestation of atypical Cogan's syndrome. A significant titer of Chalmydia trachomatis antibodies was found in our patient: their role in the pathogenesis of Cogan's syndrome is discussed.