We investigated the clinical characteristics, management modalities, and outcomes in patients with relapsing polychondritis (RP) with airway involvement. The medical records of RP patients with airway involvement seen at Samsung Medical Center from August 2004 to December 2011 were collected. The clinical manifestations were investigated retrospectively, including rheumatologic record, diagnostic tests, treatment modalities, and clinical outcomes. Twelve patients (five females, seven males) with a median age of 48(interquartile range (IQR) 44-60) years were included. All patients had airway involvement, including the trachea (100 %), main bronchi (83 %), and larynx (25 %). Rheumatological manifestations were frequent, including inflammatory arthritis (50 %), auricular chondritis (42 %), keratoconjunctivitis (42 %), nasal chondritis (42 %), saddle nose (25 %), and sensorineural hearing loss (17 %). All patients who had acute exacerbations were treated with high-dose corticosteroids (1,000 mg per day) and were maintained on oral prednisolone (5-40 mg per day), with weekly methotrexate (2.5-15 mg per week) during follow up. One out of 12 patients required mechanical ventilation. Nine patients have survived without ventilator support and eight patients without a tracheostomy. Two patients underwent a tracheostomy with endobronchial stenting. During follow-up (median 24[IQR 7-50] months), the clinical outcome was favorable in nine patients, while three patients died of pneumonia and respiratory failure. High-doses of corticosteroids during an acute exacerbation followed by maintenance prednisolone with methotrexate could be recommended as a therapeutic option in RP patients with airway involvement. Airway intervention by an experienced clinician is sometimes required.