World Health Organization (WHO) grade IV malignant glioma, including glioblastoma (GBM) with wild-type or mutant isocitrate dehydrogenase (IDH) and diffuse midline glioma (DMG) with H3K27M mutation, is the most common primary central nervous system tumor and confers a poor prognosis (1). Here, IDH3A is linked to glioblastoma.