Cogan's syndrome consists of non syphilitic interstitial keratitis associated with vestibuloauditory dysfunction. This syndrome is rare and very few cases have been reported since Cogan's first characterization in 1945. We present the case of a 24-year-oldman with a history of recurrent access of arthralgia and glomerulonephritis. Bilateral corneal disease consists of peripheral whitish patchy opacities in deep stroma. The patient also shows bilateral severe deafness, fever, meningeal and tetrapyramidal syndrome. Ocular involvement responds well to corticosteroids therapy but hearing loss is severe and often irreversible.