Objective　To understand the clinical and pathological features of Epstein-Barr virus (EBV) associated hemophagocytic syndrome.Methods　The clinical data from one deceased pediatric patient of EBV associated hemophagocytic syndrome combined with severe hepatitis were retrospectively analyzed. The relevant literatures were reviewed. Results　The 1 year and 2 months old patient presented with fever, cytopenia, decreased fibrinogen and natural killer cells, elevated serum ferritin and triglycerides. The abnormal lymphocytes accounted for 5% in blood smear. There were 1.39×108 EBV-DNA copies/ml by fluorescence quantitative detection The autopsy results suggest that the cause of death was multiple organ failure. The EB virus encoded RNAs (EBERs) were detected in liver tissues by in situ hybridization. Approximately 30% lymphocytes were EBERs positive and the hepatocytes were EBERs negative. Conclusions　The patient was confirmed to have EBV associated hemophagocytic syndrome involving multiple organs. However the liver damage was not caused by the direct infection of EB virus.