Haemophagocytic syndrome is a disorder characterised by fevers, lymphadenopathy, hepatosplenomegaly, cytopenias, and hyperferritinaemia due to dysregulated activation and proliferation of macrophages, leading to uncontrolled phagocytosis of platelets, erythrocytes, lymphocytes, and their haematopoietic precursors throughout the reticuloendothelial system. Primary or familial haemophagocytic syndrome appears to have a genetic aetiology, whereas secondary haemophagocytic syndrome may be associated with malignancy, autoimmune disease, or infection. Epstein-Barr virus is the most common infectious aetiology implicated in haemophagocytic syndrome, but the syndrome has been associated with a variety of other viral, bacterial, and parasitic pathogens. We describe a case of haemophagocytic syndrome associated with disseminated Mycobacterium tuberculosis. We review all cases of M tuberculosis-associated haemophagocytic syndrome reported in the English language literature and discuss important issues pertaining to the epidemiology, diagnosis, and management of this disease.