Cogan's syndrome is characterized by nonsyphilitic interstitial keratitis, vestibulo-auditory Menière-like symptoms, and, occasionally, systemic manifestations of vasculitis. Although neurologic manifestations are rare, several patients with stroke in the setting of Cogan's syndrome have been reported. The most common and classic ocular manifestation of Cogan's syndrome is bilateral interstitial keratitis. The diagnosis is classically suggested by the association of interstitial keratitis with acute-onset sensorineuronal hearing loss in a patient who has a negative laboratory evaluation for syphilis. Computed tomography (CT) scans may occasionally show intralabyrinthine calcifications, whereas magnetic resonance imaging (MRIs) often show soft tissue obliteration of the membranous labyrinth and may also show multiple lesions of the white matter consistent with cerebral vasculitis. The treatment of Cogan's syndrome varies based on the severity of the clinical manifestations. Due to the presumed autoimmune mechanism with vasculitis, most treatments have included steroids and immunosuppressants.