A 48-year-old woman was admitted to our hospital with respiratory failure (Hugh-Jones IV-V). She was diagnosed as relapsing polychondritis 6 years ago. Her respiratory failure was due to pharyngial stenosis, deformity and inflammation of a trachea and lobar bronchus, and bronchial collapse. Her tracheobronchochondritis was managed by 500-700 mg/day of hydrocortisone and 50 mg/day of cyclophosphamide. Laboratory examination revealed only slight elevation of CRP and no elevation of anti-type II collagen antibody, although these parameters were very high on her first admission when she had severe polyarthritis, polychondritis of nose and auricles. Bronchoscopic findings were compatible with tracheobronchomalacia since pharyngial stenosis due to inflammatory pharyngitis and bronchial collapse due to tracheobronchochondritis were shown without lung parenchymal damage. We referred to the literature on tracheobronchomalacia which was associated by the varieties of respiratory and rheumatic diseases.