Scleromyxedema is a rare chronic cutaneous mucinosis usually associated with a monoclonal gammopathy and underlying systemic disease. The etiology of the disease is not known. There are no standard treatments and response to various therapeutic modalities varies. We report a case of refractory scleromyxedema in a 63-year-old man with a biclonal IgG and IgM λ-gammopathy. The patient was successfully managed with intravenous immunoglobulin.