The high-grade glioma, H3 K27M mutant diffuse midline glioma tends to occur in children, and its high malignancy lead to very poor prognosis after treatment (median survival after diagnosis <1 year). At present, there is no standard treatment for pediatric diffuse midline glioma; surgery, conventional radiotherapy and chemotherapy are difficult to improve the prognosis. In recent years, the benefit from the stereotactic biopsy, the unique biological characteristics of pediatric H3 K27M mutant diffuse midline are being clarified. New targeted treatment methods, such as targeted chemotherapy, convection-enhanced delivery, epigenetic therapy, and chimeric antigen receptor T-cell therapy are gradually being included in the scope of clinical research. In order to clarify the current treatment progress and discuss the future goals, this article reviews recent studies about the treatment to pediatric H3 K27M mutant diffuse midline glioma.