Recent genomic studies of pediatric high-grade gliomas and DIPGs have revealed mutations in histone variant genes encoding histone proteins, Activin A receptor, type I (ACVR1) and protein phosphatase 1D (PPM1D) bringing new insights into the pathogenesis of these malignancies [4, 8, 13, 17, 18, 20, 24–26]. Here, ACVR1 is linked to malignant glioma.