In one patient, DNA methylation revealed a BCOR-ITD, usually associated with a high-grade neuroepithelial tumor, which has recently been reclassified in 2016, and reconfirmed in the 2021 WHO classification of CNS tumors as a new distinct group, previously belonging to the pediatric neuroectodermal tumor (PNET) family. The gene discussed is BCOR; the disease is central nervous system neoplasm.