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A cohort of 12 patients who developed RIG 9.5\u00a0years (3\u201331\u00a0years) after previous cranial radiotherapy for brain tumors or T-cell acute lymphoblastic leukemia was established. The derived risk of RIG development based on our consecutive cohort of 371 irradiated patients was 1.6% at 10\u00a0years and 3.02% at 15\u00a0years. Patients with RIG glioma had a dismal prognosis with a median survival of 7.3\u00a0months. We described radiology features that might indicate the suspicion of RIG rather than the primary tumor recurrence. Typical molecular features identified by molecular biology examination included the absence of <jats:italic>Histon3<\/jats:italic> mutation, methylation profile of pedHGG-RTK1 and the presence of recurrent <jats:italic>PDGFRA<\/jats:italic> amplification and <jats:italic>CDKN2A\/B<\/jats:italic> deletion. Of the two long-term surviving patients, one had gliomatosis cerebri, and the other had pleomorphic xanthoastrocytoma with BRAF V600E mutation. 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