In this study, we introduce a BCOR::CREBBP fusion in an adult patient with a right temporomediobasal tumor, for the first time in association with methylation class “CNS tumor with BCOR/BCOR(L1)-fusion” in addition to 35 cases of CNS neuroepithelial tumors with molecular and histopathological characteristics compatible with “CNS tumor with BCOR/BCOR(L1)-fusion” based on a comprehensive literature review and data mining in the repository of 23 published studies on neuroepithelial brain Tumors including 7207 samples of 6761 patients. The gene discussed is CREBBP; the disease is neoplasm.