Neurofibromatosis type 1 (NF1) is one of the most common dominantly inherited disorders. Astrocytomas, especially low-grade optic nerve tumours, are frequently harboured in these patients. In this paper, a case of a lobar cystic glioblastoma and NF1 in a 28-year-old woman is presented. This patient underwent a resection of the glioblastoma, followed a multimodal therapy including radiotherapy and chemotherapy, and survived 41 months. Neurofibromatosis is a multifaceted disease in which primary malignant CNS tumours, such as glioblastoma, can be identified. Glioblastomas in these patients should be managed like the usual ones. They may benefit from treatment with temozolomide, as can GBM patients without NF, thus potentially increasing the patient's overall survival.