Gene fusions of PLAGL1 with EWSR1 have been reported exceptionally rarely in neoplasms of the CNS, including single cases of a SMARCB1-deficient atypical teratoid/rhabdoid tumor (AT/RT) [27] and a glioneuronal tumor, not elsewhere classified (NEC) [16]. The gene discussed is EWSR1; the disease is ataxia telangiectasia.