AG-881 (Vorasidenib, Servier), as a first-in-class, brain-penetrant, dual inhibitor of the mutant IDH1 and mutant IDH2 enzymes, showed a favorable safety profile in the glioma cohort with median progression-free survival of 36.8 months [95% confidence interval (CI), 11.2–40.8] for patients with nonenhancing glioma and 3.6 months [95% CI, 1.8–6.5] for patients with enhancing glioma in a multicenter Phase I study. Here, IDH2 is linked to glioma.