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In the latest revisions of the WHO Classification of CNS Tumors, carried out in 2016 and 2021, the traditional histogenetic classification of gliomas is rebuilt on a new molecular genetic basis, according to which diffuse midline glioma with alteration of histone H3 K27 is distinguished as a specific pediatric oncosyndrome. The rationale was the association of this feature of midline gliomas in children with a worse prognosis and the prospective possibilities of targeted therapy. A thematic review of the literature and our own observation of H3 K27M-positive glioma in the area of the right thalamus in a 6-year-old child, which initially had the appearance of pilocytic astrocytoma, recurred three times (within 1.5 years) and underwent tumor progression with anaplastic transformation into diffuse glioma of the glioblastoma type, are presented. The authors are inclined to believe that although the determination of the molecular genetic status of H3 K27 provides important information regarding the probable prognosis, at the same time there is no reason to doubt that histological verification should continue to be the basis of oncological diagnosis.