The FDA approval of the IDH inhibitor, Vorasidenib, in Grade 2 gliomas harboring an IDH1 or 2 mutation based on the statistically and clinically significant randomized Phase 3 clinical trial data demonstrating prolonged progression‐free survival and time to next intervention [9], has raised the question of whether this drug could be a useful therapy in low‐grade brainstem gliomas. This evidence concerns the gene IDH2 and central nervous system cancer.