Glioblastomas in children usually occur in the brainstem and are often untreatable and associated with an extremely poor prognosis. Such tumours may however occur in supratentorial locations more typically associated with adult gliomas, where the outcome is more variable, but still poor. This report describes the case of a boy with fragile X who also developed an inoperable midbrain glioblastoma, but who has survived 8 years after diagnosis.