Hemophagocytic syndrome (HPS) associated with T cell lymphoma is well known, but an association with B cell lymphoma is rare. We report a patient who developed subcutaneous non-Hodgkin's lymphoma (NHL) of B cell origin. Autologous peripheral blood stem cell transplantation (PBSCT) was undertaken in her second complete remission (CR), but HPS appeared 5 months after transplantation. The patient deteriorated rapidly and died of metabolic acidosis. Necropsy revealed that she had relapsed B cell NHL involving liver, spleen and bone marrow. B cell NHL after PBSCT, associated with HPS as an initial sign, is rare. We report this case and review the literature.