Lymphoma-associated hemophagocytic syndrome (LAHS) is a kind of abnormal proliferation and activation of lymphocytes and macrophages induced by lymphoma or iatrogenic immunosuppression from lymphoma chemotherapy, which results excessive release of inflammatory cytokines and eventually leads to organ infiltration and tissue damage. At present, there is no universally accepted diagnostic criteria consensus for LAHS. Induction remission of LAHS through combined chemotherapy and sequential hematopoietic stem cell transplantation (HSCT) is a common therapy. This article summarizes the latest research on diagnosis and treatment of LAHS, in order to provide a reference for the clinical diagnosis and therapy of LAHS.