Relapsing polychondritis (RP) is a rare autoimmune disease of the cartilaginous structures of the body with many systemic manifestations including meningoencephalitis (ME). We present a case of a man with RP-associated ME that was responsive to steroid treatment. An updated literature review of 7 cases of RP-associated ME also is provided. Early diagnosis of this condition may be of benefit to this select population of patients, and further research regarding the prognosis, mechanisms, and treatment of RP may be necessary in the future.