Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory response syndrome induced by various pathogenic fac -tors, such as infection, rheumatic disease, and malignancy. Malignancy- associated hemophagocytic lymphohistiocytosis (MAHS) is a life-threatening disease with high rates of misdiagnosis and mortality. The diagnosis and treatment of MAHS have not been standard -ized until consensus recommendations were developed by the Study Group on Hemophagocytic Lymphohistiocytosis subtypes of the Histiocyte Society in2015. This article reviews the consensus recommendations and recent advances in MAHS.