Among these, embryonal brain tumors (EBT) represent a complex and highly aggressive group, including entities such as medulloblastoma (MB), atypical teratoid rhabdoid tumors (ATRT) and embryonal tumors with multilayered rosettes (ETMR), as well as recently described embryonal tumors with FOXR2 activation or BCOR alteration [1,2,3]. The gene discussed is BCOR; the disease is atypical teratoid rhabdoid tumor.