BCOR and central nervous system neoplasm: Herein, we present a case of a 37-year-old female with a CNS tumor harboring a pathologic BCOR c.626del (p.S209Cfs*7) (Exon 4) variant that matched the same methylation class as CNS tumors with BCOR/BCOR(L1) fusion, but without a detectable fusion, who was successfully treated with radiation followed by adjuvant temozolomide.