The presence of multiple myeloma (MM) in a patient with systemic sclerosis is a rare and unusual occurrence with unclear significance. We report the case of a 55-year-old woman with a 20-year history of systemic sclerosis, who subsequently presented with clinical stage IIIA IgG-λ MM. The systemic sclerosis was diagnosed and treated in 1988 with D: -penicillamine and methotrexate. Twenty years later, in December 2008, she presented with symptoms of Raynaud's phenomenon and intense facial pruritus. Immunoelectrophoresis confirmed the presence of a IgG-λ paraprotein (71.90 g/l) and Bence Jones proteinuria of the lambda light chains. Bone marrow histology revealed infiltrates of plasmocytes and lymphoplasmocytes which were on immunohistochemistry CD38+, FGF-R3+ and OPG+. An extensive X-ray skeletal survey did not show any osteolytic lesions or fractures. The patient was treated according to the CTD protocol (cyclophosphamide, thalidomide, and dexamethasone) which was effective against the myeloma as well as the systemic sclerosis and patient achieved complete remission.