Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a rapidly progressive disease with a high rate of mortality. In the patients with EBV-HLH, hypercytokinemia plays a major pathogenetic middle mechanism. The clinical manifestations were various and its diagnosis should meet HLH 2004 criteria as well as EBV-positive. In terms of treatment, the combination of dexamethasone, etoposide and cyclosporin A is the first choice. If they do not work，hematopoietic stem cell transplantation can be done. The recent progress on diagnosis and treatment of EBV-HLH is reviewed in the present paper.