Tuberculosis associated hemophagocytic syndrome (HPS) has recently been recognized as a benign reactive histiocytic proliferation with marrow hemophagocytosis. To our knowledge, only five autopsy documented cases have previously been reported. We present here a unique case of the disorder complicated by severe bone marrow failure and disseminated intravascular coagulation. The possible mechanisms of these complications are discussed and it is concluded that the immunological disturbances usually occurring in miliary tuberculosis could play a role in the pathogenesis of HPS.