Schwannomas arising solely from the cochlear nerve and limited to the internal auditory canal are rare. Only three prior cases have been specifically described in the literature. We report a 38-year-old male with and asymmetric audiogram and poor discrimination. Magnetic resonance imaging revealed a 3-mm mass occupying the inferior portion of the internal auditory canal. Discrimination improved following a course of steroids, but thresholds did not. The patient underwent a translabyrinthine removal of the tumor, which was confirmed to involve only the cochlear nerve. It is important to obtain evidence regarding the possibility that a tumor confined to the internal auditory canal is a cochlear schwannoma. If so, surgery can be deferred until hearing is no longer functional or tumor growth mandates removal.