Upon review of LZTR1 findings in our childhood (non-ependymoma) cancer control cohort, the LZTR1 missense VUS (p.Asp703Asn [c.2107G > A]) detected in another patient with PF-EPN-A1c was observed in a child with acute myeloid leukemia. The gene discussed is LZTR1; the disease is ependymoma.