Relapsing polychondritis (RP) is a rare immune-mediated systemic inflammatory disease with diverse clinical manifestations. Independent involvement of the respiratory system in RP is uncommon. In the event of respiratory involvement as the initial airway-only manifestation, the diagnosis of RP is challenging and might be delayed, and patients with respiratory involvement exhibit a poor prognosis. However, no specific diagnostic method is currently available for RP with respiratory system involvement as the main clinical manifestation. We present a 49-year-old female with the complaint of chronic dry cough accompanied by shortness of breath after exercise that has persisted for over a year. The patient was treated using corticosteroids. The patient's symptoms improved rapidly with the administration of 5 days of methylprednisolone sodium succinate at a dose of 40 mg/day. The treatment was then switched to methylprednisolone tablets at a dose of 40 mg/day, and the dosage was reduced by 4 mg every week until the cessation of therapy. Meanwhile, oral cyclophosphamide tablets were administered once every day at a dose of 100 mg each time. After 1 month of treatment, the symptoms of cough disappeared, the modified british medical research council (mMRC) grade dropped from 4 to 2, and the COPD assessment test (CAT) score dropped from 30 to 17. Repeated CT of the chest revealed that the tracheal wall thickening had alleviated. No recurrence was revealed in the follow-up visit 12 months after drug withdrawal. The patient underwent