After the first description of CNS tumor with BCOR ITD [8], other tumors with overlapping morphological and immunohistochemical features, but harboring fusions of BCOR with either EP300 or its paralog CREBBP, instead of BCOR ITD, have been reported in the CNS [7, 9, 11, 12, 14, 15]. This evidence concerns the gene EP300 and central nervous system neoplasm.