Hemophagocytic Iymphohistocytosis is severe disease in children, also a big challenge of pediatricians. Epstein-Barr virus-associated hemophagocytic Iymphohistiocytosis (EBV-HLH), also known as EBV-associated hemophagocytic syndrome, develops mostly in children and young adults and may be fatal. The authors presented a case with Epstein-Barr virus - associated hemophagocytic Iymphohistiocytosis. EBV copies is very high in blood when the patien admitted. The patient had six of eight Criteria to diagnose HLH, she had not been treated by protocol HLH yet even included Acyclovir IV or IVIG or corticoid. She was only treated by antibiotic, plasma, human albumin. She started recover after 4 days admission (on 14th of her disease) and recovered completely after 1 month from onset.