METHODS A case of a 54-year-old woman with a three month history of recurrent bilateral chondritis of the auricles, cochlear and vestibular inner ear damage and conjunctivitis is described. The diagnosis was based only on clinical criteria (auricular chondritis, conjunctivitis, inner ear damage). Antinuclear antibodies, ANCA and HLA-DR 4 antigen were negative. The only laboratory abnormality was an elevated erythrocyte sedimentation rate. The patient has been treated successfully with Methotrexate 20 mg 1 x /week and Prednisone 15 mg/die for 4 month now. CONCLUSIONS The relapsing polychondritis (RP) is a rare, multisystemic and potentially fatal disease. The pathogenesis and optimal therapeutic approach is poorly understood. The disease is characterized by episodic inflammation of cartilage such as auricular, nasal and laryngotracheal. Many other proteoglycan-rich structures like inner ear, eye, kidney and blood vessels, may be involved as well. RP has an equal sex prevalence. The majority of cases appear between 40 and 60 years. Auricular inflammation is the most common feature. Effectiveness of non-steroidal anti-inflammatory drugs, dapsone, immunosuppressive drugs and prednisone has been described. The overall survival rates were 74 % at 5 years and 55 % at 10 in one 1986 series. CONCLUSIONS The most common clinical presentation of RP regularly involves ENT-structures. Therefore ENT-specialists should be familiar with this disease. A close interdisciplinary cooperation is essential for therapy and follow-up, because pulmonary and cardiac involvement are limiting prognostic factors.