NTRK fusions have been identified in several types of pediatric gliomas, with an incidence of around 5% in pediatric high-grade gliomas and glioblastomas.6BEND-5 gene alterations have rarely been reported in adult central nervous system (CNS) tumors such as glioblastoma but have not previously been reported in pediatric PXA2 or PXA3.7 We report a patient with a history of cranial radiation for rhabdomyosarcoma (RMS) who developed a PXA3 with BEND5-NTRK2 fusion 20 years later. Here, NTRK2 is linked to glioblastoma.