Abstract: Mutation of the isocitrate dehydrogenase (IDH) gene is a critical driver event in diffuse gliomas. By catalyzing the production of an abnormal metabolite, 2-hydroxyglutarate (2-HG), IDH mutation triggers a series of molecular alterations, including abnormal DNA and histone methylation, metabolic reprogramming, and DNA repair defects, thereby providing a theoretical basis for targeted intervention. Currently, the treatment of IDH-mutant gliomas is gradually transitioning toward molecularly oriented precision therapy. This article reviews the epidemiological characteristics, core molecular mechanisms, and stratified standard treatment strategies of IDH-mutant gliomas, with a focus on collating clinical evidence of emerging therapeutic approaches such as IDH inhibitors, PARP inhibitors, and immunotherapy, so as to provide references for the precise diagnosis and treatment of this type of tumor.