Relapsing polychondritis is a rare systemic disease. Clinical manifestations are variable and the condition is frequently associated with other diseases. Chondritis typically involves ears, nose, costal cartilages and upper airways. Other classical features include scleritis and episcleritis, mucocutaneous lesions, arthralgia and constitutional symptoms. Repeated inflammation of cartilaginous structures may lead to deformities of the ears, the nose and the airways. Tracheobronchomalacia and aortitis of the ascending portion resulting in aortic regurgitation or aneurysm are feared complications. Treatment is mainly based on systemic corticosteroids alone or in association with immunosuppressants. Several biological immunosuppressive agents are reported effective in refractory disease.