Objective: To improve understanding of the accelerated phase of chronic myeloid leukemia (CML-AP) in children with EVI1 gene positivity. Method: A retrospective analysis was conducted on the clinical data of one EVI1 gene positive child CML-AP patient admitted to Qingdao Women and Children's Hospital in January 2019, and relevant literature was reviewed. The patient, male, 14 years old, was diagnosed and received a combination of domestic imatinib and low-dose chemotherapy, achieving complete hematological remission. The quantification of BCR-ABL p210 decreased to 4.66%, and the quantification of EVI1 decreased to normal (0.01%). Conclusion: CML-AP in children is rare, and its clinical features mainly include a significant increase in white blood cell count and splenomegaly. The combination of domestic imatinib and low-dose chemotherapy is effective in the treatment of CML-AP in children with EVI1 gene positivity, but the long-term efficacy still needs close follow-up.