Scleromyxedema is a rare skin disease associated with paraproteinemia and characterized by the deposition of mucin in the skin. The pathogenesis of scleromyxedema is unknown. Although scleromyxedema is limited to the skin in most cases, cases with systemic involvement has been reported in the literature. Scleromyxedema has significant morbidity and mortality and is often difficult to treat. Despite reports of success with different agents, no satisfactory treatment is currently available. In this report, we present a patient with scleromyxedema who was treated with different agents without any improvement and successfully treated with high-dose intravenous immunoglobulin (hdIVIg).