The presence of a monoclonal gammopathy in patients with chronic scleredema is a recently described, unusual association of unknown significance. There have been very occasional reports of multiple myeloma in such patients. We now report a patient with long-standing scleredema who developed an IgA-kappa multiple myeloma. Once chemotherapy for the myeloproliferative disorder was instituted, scleredema improved significantly, suggesting a pathogenetic link between the two conditions.