Relapsing polychondritis (RP) is rare, chronic, relapsing, and multisystemic inflammatory disease targeting the cartilaginous structures. Respiratory track involvement occurs in approximately half of the cases. Subglottic stenosis is a rare manifestation of RP. Here, we report a case of RP with a subglottic stenosis, resulting in acute respiratory failure. A 63-year old man was admitted complaining of multiple joint pain, general weakness, weight loss, throat pain, hoarseness, exertional dyspnea, and hearing difficulties. A laryngoscopy and neck CT revealed a subglottic stenosis. Four days after admission, he complained severe dyspnea resulting in acute respiratory failure. Immediately, a tracheostomy was done for airway preservation. After high dose steroid therapy, the general symptoms were improved. However, the subglottic stenosis was sustained. Thus, a laryngotracheal augumentation and stent insertion was performed. The speech valve was then replaced. The subglottic stenosis was managed with low dose steroid and monthly cyclophosphamide pulse therapy, and the patients has been followed up regularly.