A male patient had suffered miscellaneous ocular symptoms for 20 years with auricular dysmorphosis and was diagnosed with Relapsing Polychondritis (RP) in the ear, nose, joints, and costal cartilage. The patient lost his vision owing to recurrent ocular symptoms for decades. He presented an increased IgA and was diagnosed with monoclonal gammopathy of undetermined significance (MGUS) and treated by prednisone and cyclophosphamide. His ocular symptoms relieved and serum IgA decreased after six months. In conclusion, RP is a systemic disease with a wide range of clinical symptoms and may lead to serious complications.