IntroductionNearly 40% of pediatric CNS tumors are low-grade gliomas (LGGs), a subgroup of heterogeneous, slow-growing tumors.(1) In fact, pediatric intramedullary spinal cord LGGs are very rare.(2) Recently, tumor diagnosis and prognostic evaluation, as well as therapeutic management, have been addressed by molecular profiling, which become significant for optimal patient management.(3–7) Moreover, there is recent attention on the neurotrophic tropomyosin receptor kinase (NTRK) gene alterations, NTRK is a family of genes (NTRK-1, NTRK-2, and NTRK-3) encoding for the tropomyosin receptor kinase (TRK-A, TRK-B, and TRK-C).(8) NTRK gene plays a role in the growth, differentiation, and survival of neurons. (9,10)NTRK fusions have been known to be involved in many adolescent and pediatric cancers.(11) However, NTRK-2 fusion is majorly involved in pediatric patients.(12) It should be noted that only less than 1% of tumors are thought to contain NTRK fusion (13,14) and 0.55 to 2% of gliomas/neuroepithelial tumors contain NTRK.(13–18) Despite the rarity of our present case, the literature highlighted the potential clinical benefits of using targeted therapy (e.g., Larotrectinib) in tumors containing NTRK fusion.(19,20) Larotrectinib (LOXO-101) is a highly selective inhibitor of TRKA, TRKB, and TRKC administered orally.(21) Though, the efficacy of Larotrectinib has not been sufficiently defined yet.In this study, we report a case of pediatric intramedullary spinal cord low-grade gliomas with NTKA fusion, a long (fourteen-month) follow-up. Our patient provided a consent form and agreed to the publication of this report.