Cogan's syndrome is an inflammatory disease that is characterized by ocular inflammation (typically interstitial keratitis) and is associated with Ménière-like vestibuloauditory dysfunction. Ocular inflammation usually resolves after several weeks or months but deafness is often irreversible. We report on a case of Cogan's syndrome in a 23-year-old woman who initially presented with bilateral anterior uveitis, an unusual clinical feature for this disease. We discuss the clinical aspects, the pathogenic mechanisms, the laboratory investigations, the differential diagnosis, and the treatment of Cogan's syndrome.