Relapsing polychondritis (RPC) is a chronic immune-mediated inflammatory, systemic disease primarily leading to structural damage and impaired function of cartilage tissue. However, the systemic inflammatory process in RPC can also affect sensory organ structures, the respiratory tract, the nervous and cardiovascular systems as well as the kidneys. The immune-mediated disease leads to recurrent inflammatory attacks causing a progressive degradation of elastic and hyaline cartilage structures, especially in the ears, nose, larynx, trachea and diarthrodial joints. However, other connective tissue structures in the eye and the heart valves may also be involved. The RPC is regarded as an orphan disease as the number of reported cases has so far remained confined to approximately 600 worldwide. The rarity of the disease has limited systematic clinical studies and the available empirical data are exclusively derived from casuistic studies or evaluation of small case series. The therapeutic interventions depend on the extent and localization of the disease manifestation. Thus, nonsteroidal anti-inflammatory drugs (NSAID), glucocorticoids and immunosuppressive agents with conventional synthetic disease-modifying antirheumatic drugs (DMARD) have been demonstrated to be beneficial. More severe and refractory diseases may require a targeted pharmacological intervention with biologic DMARDs.