Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a severe life-threatening clinical syndrome with hyperinflammation rather than an isolated disease.It is classified as primary HLH and secondary HLH.Primary HLH is associated with HLH-related gene mutations,whereas secondary HLH occurs after severe infections,tumors,connective tissue diseases,etc.[1] Regardless of primary or secondary HLH,30-73% of patients have central nervous system (CNS) involvement.[2] Among adults with HLH in China,38.4% of patients have neurological symptoms at the onset,and there are up to 89.6% with neurological symptoms during the entire course of the disease.[3] However,it has not been clearly reported about the CNS involvement in children with HLH.