Science and Education Publishing American Journal of Medical Case Reports 2374-216X 2 7 2014 07 27 Atypical Cogan's Syndrome: Case Report of an Oculoaudiovestibular Disease 139 142 EN Btissaam. Belhoucha Department of ENT, CHU Med VI, Marrakech 2360, Morocco S Belghmidi I. Benbaddan Youssef. Rochdi I Hajji Hassan. Nouri lahcen. Aderdour M. Hattaoui A Moutaouakil Abdelaziz. Raji AJMCR2014273 10.12691/ajmcr-2-7-3 2014 06 30 2014 07 21 2014 07 27 Cogan's syndrome (CS) is a rare autoimmune vasculitis. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between Cogan's syndrome and systemic vasculitis as well as aortitis also exists. We report a case of cogan's syndrome in 12 year old girl who intialy present with bilateral interstitial keratitis with audiovestibular and cardiac symptoms. We discuss the clinical aspect, the pathogenic mechanisms, the laboratory investigations, the differential diagnosis and traitement of cogan syndrome.