Relapsing polychondritis is a unique, rare autoimmune disorder in which the cartilaginous tissues are the primary targets of destruction but the immune damage can spread to involve noncartilaginous tissues like the kidney, blood vessels, and so forth. The manifestations of the disease can take many different forms and the pathogenesis is still unclear. It may occur in a primary form or it may be associated with other disease states. This article summarizes important aspects of the disease with a focus on recent information regarding clinical manifestations, disease associations, pathogenesis, and advances in therapeutics.