Abstract: Hemophagocytic syndromes also known as hemophagocytic lymphohistiocytosis (HLH), is a rare and life-threatening clinical syndrome. HLH is caused by the proliferation and activation of lymphocytes and macrophages, which secrete cytokines. The excessive secretion of cytokines leads to damage of multiple organs and systems. Immunotherapy is an important part of the treatment of tumors and autoimmune diseases, which has changed the traditional treatment mode and improved the survival rate of patients. Immunotherapy-related HLH has attracted the attention of researchers due to its severity and rapid progression. At present, this type of HLH was usually reported as case, lacking uniform diagnosis and treatment standards. This paper will summarize the pathogenesis, diagnosis and treatment progress of immunotherapy-related HLH in order to improve the knowledge of medical practitioners about this disease.