To analyze the importance of airway involvement in relapsing polychondritis, an illustrative case report is presented and 62 patients reported in the literature with serious airway complications are reviewed. There were 47 female and 17 male patients, with an average age of 40.3 years (range, 2 to 73 years). Patients were seen with hoarseness, breathlessness, cough, stridor, wheezes, and tenderness over laryngotracheal cartilages. Respiratory tract involvement was confirmed by conventional radiography, tomography, computed tomography, dynamic pulmonary function tests, and bronchoscopy. Corticosteroids and antiinflammatory and immunosuppressive agents were used in these patients. Tracheostomy was performed in 18 patients. Death occurred in 13 patients despite tracheostomy or corticosteroid therapy, or both. A detailed analysis of the clinical, radiological, and pulmonary function studies is presented, with emphasis on upper airway mechanics. The medical and surgical management options are reviewed, including the use of endotracheal prosthesis and extraluminal splinting in dynamic airway collapse.