In this article, we report on a 40-year-old man with relapsing polychondritis (RP) of the tracheobronchial tree without clinical manifestations of other systems including nasal septum and earlobe cartilage involvement. The illness was diagnosed histologically, and treatment with procaine penicillin was successful. RP is an unusual systemic disorder characterized by widespread inflammation and destruction of cartilage tissues. The main cause is usually autoimmune. In RP various clinical manifestations including nasal chondritis, arthritis, scleritis, damage to tracheal and bronchial cartilage, and aortic, cardiac, and renal involvement may occur. Isolated tracheobronchial involve ment is very rare. The diagnosis must be based on a combination of clinical and pathologic features. If diagnosed early, appropriate treatment may prevent life-threatening airway obstruction.