Ependymoma, RELA fusion-positive (EPN-RELA) was newly proposed in the WHO Classification of Tumours of the Central Nervous System (CNS), 2016 updated fourth edition (CNS4) [1] as an ependymoma (EPN) with a C11orf95-RELA fusion gene typically occurring in the supratentorial compartment. This evidence concerns the gene RELA and ependymoma.