Tuberculosis is a common infection in Taiwan, and it is probably a cause of reactive hemophagocytic syndrome. We report the case of a 63-year-old man with initial presentation of fever and progressive jaun-dice. Hemophagocytic syndrome was documented by the findings of peripheral blood smear and bone marrow biopsy. Although chemotherapy and antituberculous therapy were administered early, he passed away. Sputum and bone marrow cultures confirmed the presence of Mycobacterium tuberculosis 3 weeks later. Bone marrow biopsy revealed noncaseating granuloma. Patients with hemophagocytic syndrome should be rigorously screened for tuberculosis and antituberculous therapy should be initated early to improve prognosis.