Central nervous system manifestations are rare in patients with relapsing polychondritis. We report a case of encephalitis associated with relapsing polychondritis. The patient presented with bilateral auricular chondritis first and then tracheal chondritis, and encephalitis developed. The symptoms and signs of encephalitis were felt mostly likely to be the result of vasculitis and improved dramatically after pulse corticosteroid therapy. We believe that early recognition of central nervous system involvement in relapsing polychondritis is extremely important. High-dose corticosteroids should be given to suspected cases as early as possible.