We present an unusual tumorous variety of scleromyxedema mimicking facies leonina in lymphoma. In spite of pronounced and widespread cutaneous changes,hypergammaglobulinaemia and paraproteinaemia, the general condition of the patient was satisfactory, there was no internal involvement and no symptoms of any malignanc y. Initially, melphalan and corticosteroids were applied but were not effective. High-dose intravenous immunoglobulin (IVIG) therapy had dramatic effect, and a fter five 5-day monthly courses the tumours almost regressed and the skin becam e less hard. After a further five courses in the following year there was comple te clearance, which was sustained without any therapy for 1 year (until now). IV IG appears to be the therapy of choice for scleromyxedema. We stress, however, t hat at the start of therapy, IVIG applications should be supplemented with small doses of melphalan and/or corticosteroids. We present an unusual tumorous variety of scleromyxedema mimicking facies leonina in lymphoma. In spite of pronounced and widespread cutaneous changes, hypergammaglobulinaemia and paraproteinaemia, the general condition of the patient was satisfactory, there was no internal involvement and no symptoms of any malignanc y. Initially , melphalan and corticosteroids were applied but were not effective. High-dose intravenous immunoglobulin (IVIG) therapy had dramatic effect, and a fter five 5-day monthly courses the tumours almost regressed and the skin becam e less hard. After a further five courses in the following year there was comple te clearance, which was sustained without any therapy for 1 year (until now). IV IG appears to be the therapy of choice for scleromyxedema. We stress, however, t hat at the start of therapy, IVIG applications should be supplemented with small doses of melphalan and / or corticosteroids.