Relapsing polychondritis is a rare autoimmune disease, which is due to sporadic occurrence, unexplained etiology and wide range of symptoms resulting in the difficulties in diagnosis. Recurrent inflammation leads to destruction of cartilaginous tissues, such as ear, nasal septum, larynx, trachea and bronchi, peripheral joints and vertebral structures. Blood vessels, the central nervous system, eyes, and the urinary tract can be involved as well. Clinical course of relapsing polychondritis is often modified by co-existing systemic diseases and remains misdiagnosed. In case of inappropriate or delayed treatment, serious and life-thretening complications may develop. In this article we present the most characteristic clinical features of the disease, differential diagnosis, focusing on current approaches to treatment.