Objective　To explore the diagnosis and treatment of essential thrombocythemia in children. Methods　The clinical data from 8 children with essential thrombocythemia were retrospectively analyzed. Results　From January 2010 to June 2015, there were 8 children diagnosed essential thrombocythemia in clinic. One child was only 10 months old. Among them, 6 cases had JAK2V617F mutation, 2 cases had no mutation. All children were treated with hydroxyurea or interferon-α, and achieve remission. Conclusion　Essential thrombocythemia is a rare disease in childhood with a growing trend. More children with essential thrombocythemia should be enrolled for comparison of clinical manifestations and prognosis between JAK2V617F mutation positive and negative cases.