A patient with primary plasma cell leukemia is described. Immunoelectrophoresis revealed a monoclonal IgG/λ protein and Bence Jones protein. Hematological data showed numerous immature plasma cells in peripheral blood and bone marrow. Cytogenetic study of tumor cells revealed a complex karyotypic abnormality. Subcutaneous involvement by tumor cells was found. The patient was treated with a combination of vincristine, doxorubicin, dexamethason (VAD) and achieved a complete remission with disappearance of tumor cells, monoclonal protein and extramedullary involvement after 6 cycles of VAD. This regimen should be encouraged in treatment of primary plasma cell leukemia.