Keratitis-ichthyosis-deafness (KID) syndrome is a rare ectodermal dysplasia characterized by vascularizing keratitis, profound sensorineural hearing loss (SNHL), and progressive erythrokeratoderma. It is a clinical triad that indicates a failure in development and differentiation of multiple stratifying epithelia. KID syndrome has been associated with mutations in the gene encoding connexin 26, a gap junction protein. The aim of this case review was to investigate auditory performances after cochlear implantation and emphasize the importance of auditory rehabilitation. The results of this case suggested that auditory performances and communication skills of a child with KID syndrome have improved through the cochlear implantation and aural rehabilitation. In particular listening tests of Listening Progress Profile (Lip Profile) and Meaningful Auditory Integration Scale (MAIS), the scores have improved from 52% to 97% and 20% to 80%, respectively. The results of this study suggested that auditory rehabilitation and cochlear implantation in children with (KID) syndrome can be effective and important. KEY WORDS：Aural rehabilitation·Cochlear implantation·Keratitis-ichthyosis-deafness (KID) syndrome.