Scleromyxedema is a rare chronic skin disorder of unknown etiology, often accompanied by monoclonal gammopathies. Clinically the disease is characterized by generalized thickening and stiffening of the skin due to dermal mucin deposition. We report on a 56-year-old women with scleromyxedema, whose skin findings markedly improved following high-dose intravenous immunoglobulin therapy (Sandoglobulin((R)), 2 g/KG). The pronounced clinical benefit was documented by increased flexibility of the fingers, reduction of skin induration, and a decrease in number and size of mucinous papules. All previous therapeutic approaches were ineffective. High-dose intravenous immunoglobulin appears to be a promising treatment option for patients with scleromyxedema. Clinical studies are desirable, but difficult to perform in such a rare disease. Long-term follow-up on individual patients so treated is desirable.