ObjectiveTo summarize the clinical manifestations,diagnosis and treatment of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis. MethodsOne proven diagnosed case of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis was analyzed. Related publications of case reports and articles from Wanfang Data and China Hospital Knowledge Database were also reviewed. ResultsA total of 15 cases were reported,in which 9 cases with complete clinical data. There were 7 males and 2 females,with a mean age of 31.2±15.83 years. The infected tissues were as follows:lung(6 cases),liver(2 cases),spleen(1 case),lymph node(2 cases),pleural(3 cases),peritoneum(2 cases),meninges(1 case),two or more sites infection(5 cases). The most clinical manifestations were fever,hepatosplenomegaly and enlarged lymph glands. The common laboratory abnormalities were liver dysfunction,pancytopaenia and haemophagocytosis in bone marrow. Treatment and outcomes:immunotherapy(5 cases,3 cases with intravenous immunoglobulin and 5 cases with steroid therapy) and anti-tuberculosis (14 cases). 3 cases were cured,6 cases improved and 6 died. ConclusionThere were no specific indexes of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis. Early proven diagnosis and administration of anti-tuberculosis and immunotherapy is essentials of effective therapy.