Coexistence of scleroderma with multiple myeloma (MM) is an unusual finding with unclear significance. Only 13 cases of MM with scleroderma have been reported until now. We report a case of a 24-year-old man with 8-year history of progressive thickening of skin all over the body. Histopathology of skin lesion was consistent with scleroderma. Bone marrow biopsy showed interstitial and focal increase in plasma cells and increased bone marrow fibrosis. Skeletal survey showed osteopenia, but no osteolytic lesion or fracture. The patient was diagnosed as scleroderma with coexistence of immunoglobulin A, κMM. The patient recovered with improvement of skin lesions after 9 months of therapy with thalidomide and dexamethasone.