Cogan's syndrome is characterized by non syphilitic interstitial keratitis and Meniere's syndrome-like audiovestibular function disorder, as well as various systemic manifestations, including fever, arthralgia, vasculitis, and aortitis. We report the case of atypical Cogan's syndrome with aortitis; the patient's symptoms caused by active inflammation, including inflammatory ocular manifestations, audiovestibular symptoms, and aortitis, were improved by early administration of corticosteroids, however, her reduced visual acuity was not improved because of post-inflammatory change in her left eye.