Abstract: 　Objectives　To explore the clinical presentation, diagnosis and treatment of hemophagocytic syndrome (HLH) in children. Methods　The clinical data from 11 hospitalized pediatric patients with HLH were collected and retrospectively analyzed from 2009 to 2013. Results　In 11 pediatric patients with HLH, 6 patients were Epstein-Barr virus associated-HLH, 1 patient was T-cell lymphoma associated-HLH, 2 patients had unknown reason, 2 patients had mutations in the UNC13D gene coding sequence, c.2459C>T/p.A832V (alanine to valine mutation) and c.3067C>T/p.R1023C (arginine to cysteine mutation) respectively. In 11 patients, 6 patients were improved after treatment and 5 patients were died. Conclusions 　The HLH in children lacks specific clinical presentation and progresses rapidly. It should be diagnosed and treated in time.