In recent years，with the deepening understanding of the genetic characteristics and molecular markers of childhood acute myeloid leukemia（AML），researchers have identified potential pathogenic mechanisms related to relapsed or refractory（R/R）AML.This discovery has promoted the development and application of targeted therapeutic drugs for AML and is expected to improve the prognosis and therapeutic efficacy for children with R/R AML.This article reviews four classes of drugs that have made progress in the targeted therapy of R/R AML in children：gene-targeted therapies，signaling pathway targeting，targeting of cell surface antigens，and epigenetic inhibitors，with the aim of providing useful references and insights for clinicians and researchers.