Together with the previous report of a H3 K27M‐mutant midline glioma in association with MUTYH mutation,2 our H3 K27M‐ and H3 G34V‐mutant cases suggest a potential link between pediatric gliomas and MUTYH mutations and expand our knowledge on the pathogenesis of these tumors. The gene discussed is MUTYH; the disease is glioma.