Our case is of special interest because, for the first time, Arg140Gln is documented in a glioma and occurred in a lesion with hybrid molecular features between astrocytoma and oligodendroglioma (with an absence of the ATRX variant, presence TP53 variants, and no 1p–19q codeletion) (Figure 1). The gene discussed is ATRX; the disease is central nervous system cancer.