This case, which has been previously published (21), demonstrates strong similarities regarding clinico-radiological, histopathological, immunohistochemical, and methylome features with HGNET-BCOR with ITD but its classification according to the c-IMPACT-NOW update 6 (15) will be difficult since the terminology of “CNS tumor with BCOR-ITD” cannot be used in the absence of BCOR-ITD. Here, BCOR is linked to central nervous system neoplasm.