We report the case of a 41-year-old female patient with relapsing polychondritis and severe respiratory involvement. The patient presented with acute respiratory failure requiring endotracheal intubation. Bronchoscopy revealed tracheal collapse and inflammatory stenoses with dynamic collapse of the major airways. We describe a multidisciplinary therapeutic approach, consisting of immunosuppressive treatment, bronchoscopic placement of self-expandable stents into the collapsing bronchi, and tracheotomy. In addition, we report the effectiveness of oral cyclophosphamide for treatment of relapsing polychondritis with severe respiratory involvement after failure of other immunosuppressive agents. The problem of severe respiratory complications in patients with relapsing polychondritis and the need for a multidisciplinary approach is discussed.