Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease characterized by uncontrolled cytotoxic T cells and macrophages. Contributing causes include heredity, infection, autoimmune disease, and tumors, which stimulate the activation of macrophages and secretion of a large number of inflammatory factors. Most malignancy-associated hemophagocytic lymphohistiocytosis (MA-HLH) patients are in critical condition at time of diagnosis, due a high rate of delayed diagnosis and misdiagnosis, resulting in highmortality. With extensive application of immune checkpoint inhibitors, incidence of MA-HLH is increasing. This article reviews recent research progress in this field, focused on epidemiology, predisposing factors, pathophysiology, diagnostic criteria, and treatment.