We present two patients with acute myeloblastic leukemia (FAB AML-M7 and AML-M2) complicated by hemophagocytic lymphohistiocytosis (HLH) and relevant literature review. To our knowledge, our first case is the first and youngest patient having AML-M7 associated with HLH reported in the literature. Our cases and cases in the literature highlight the high mortality rate of leukemia associated with HLH and the need for further investigation for the most appropriate therapy.