Pediatric diffuse intrinsic pontine gliomas (DIPGs) belong to the most aggressive and deadliest childhood brain tumors classified as histone H3 Lysine 27 (H3K27)-altered diffuse midline gliomas (DMG), including the H3 K27-mutant as well as H3 wildtype subtypes with overexpression of the EZH inhibitory protein (EZHIP) [1,2,3]. Here, EZHIP is linked to diffuse midline glioma.