Gliomas are a type of brain tumor that can be challenging to diagnose and treat, especially in children. Diffuse midline glioma (DMG) is a newly defined subtype that has a very poor prognosis. The diagnosis of DMG is based on clinical presentation and characteristic features on conventional magnetic resonance imaging (MRI). However, advanced MRI and positron emission tomography (PET) imaging can provide additional information about the tumor's molecular and metabolic features. In this study, the authors summarize the metabolic pathways of DMG and their imaging surrogates to explore the potential of advanced imaging techniques for improving the diagnosis and treatment of this devastating disease.