Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype (pHGG H3/IDH WT) represents a heterogeneous group of tumors, which is characterized by histological high-grade features, absence of histone H3 and IDH mutations, and aggressive biological behavior (CNS WHO grade 4) (WHO Classification of Tumours Editorial Board, 2021). Here, IDH1 is linked to glioma.