Central nervous system involvement by relapsing polychondritis is rare. Despite the fact that several reports described its cognitive presentations, the detailed neuropsychological characteristics have not been provided. Severe cognitive impairment was noted in our patient with meningoencephalitis due to polychondiritis. With immunosuppressant therapy, his deficits recovered markedly, but not completely in some severely affected cognitive domains from the earlier time. Neuropsychological complications of polychondritis are very responsive to immunosuppressants although it does not seem to be perfect.