Abstract: Diffuse midline glioma (DMG), most frequently occurring in children, is a fatal malignant tumor that occurs at the midline position and is mainly characterized by mutations at lysine 27 in histone H3 (such as K27M). Diffuse intrinsic pontine glioma (DIPG) is a type of DMG that occurs in the pons. Surgical and chemotherapeutic intervention outcomes are suboptimal. Radiotherapy is currently the only standard treatment in clinical practice that can improve symptoms and delay tumor progression. Stereotactic intracranial biopsy can safely and reliably provide tissue for clinical molecular typing and diagnosis of DMG, offering an important pathway to more detailed tumor typing and development of new approaches to precise targeting of therapeutic regimens based on radiotherapy and immunotherapy. Immunotherapy is an emerging treatment method that eliminates tumor cells by stimulating the patient's immune system, offering advantages of precision and minimal side effects. Remarkable recent achievements have been made in DMG treatment, including adoptive immunotherapy, oncolytic viral therapy, vaccine therapy, immune checkpoint inhibitor therapy, and combination therapy. This article reviews the latest research progress in DMG immunotherapy and elaborates on the action, clinical effects, and remaining challenges facing each therapy, aiming to provide new ideas and methods for clinical treatment of children with DMG and promote transformative clinical application of immunotherapy to DMG treatment.