We report a 70-year-old male case of immunoblastic lymphadenopathy-like T-cell lymphoma (IBL-T) complicated by hemophagocytic syndrome (HPS) as a terminal event. The patient experienced fever and systemic lymphadenopathy after 22 months' remission of IBL-T. He developed acute hepatic failure and consumption coagulopathy rapidly, and died on the 7th hospital day. Serum levels of cytokines, including interferon-gamma and tumor necrosis factor-alpha, were elevated. Postmortem bone marrow aspirate showed a marked proliferation of benign-looking macrophages, some of which phagocytized erythrocytes and platelets. Because there were no preceding viral or bacterial infections, the HPS of this case was assumed to be associated with IBL-T at relapse.