Polychondritis recidivans is a rare immune-mediated autoimmune, multisystemic disease. It proceeds with recurrent inflammation of both cartilage tissue, mainly the ears, nose, larynx, tracheobronchial tree, ribs and joints, as well as the proteoglycan-rich tissues of the heart valves, blood vessels and eyes. The clinical picture is diverse. Symptoms often overlap with other diseases and diagnosis is delayed. Late diagnosis is the main cause of complications, and life-threatening damage to the affected structures. Polychondritis recidivans may be self-contained or associated with other autoimmune diseases, vasculitis, or hematological disorders. The diagnosis is clinical. The main manifestation of diagnostic value is chondritis with sequential or simultaneous involvement of several cartilage organs. There is no specific laboratory test for the disease. A cornerstone of therapy is glucocorticoids. With severe course, immunosuppressants are also used, and recently biological agents. We describe a case of a 51-year-old woman with repeated recurrences of moving inflammation of the ear cartilage, accompanied by arthritis, severely reduced hearing, hoarseness, conjunctivitis and episcleritis, and CNS symptoms. Blood tests showed leukocytosis and elevated C-reactive protein. Polychondritis recidivans is diagnosed based on the diagnostic criteria of L. P. McAdam et al., J. M. Damiani and H. L. Levine and C. J. Michet et al. Dramatic improvement from methylprednisolone treatment supported the diagnosis. An overview of the epidemiology, pathogenesis, clinical picture, complications, laboratory and imaging diagnostic methods, and treatment of polychondritis recidivans are presented.