We report on a patient who presented at our hospital with fever, headache, neck pain, partial nuchal rigidity and decreased vision of the left eye. The clinical history, biochemical and instrumental exams performed suggested meningitis but the final hypothesis achieved was an unusual case of Neuro-Behcet-Disease (NBD) without orogenital ulcerations at presentation and with normal MRI findings, whose course was complicated by fatal cerebral venous sinus thrombosis and intracranial haemorrhage. The post-mortem results confirmed the diagnosis. This is a rare case confirmed by anatomo-pathological findings where NBD can present itself as an acute meningeal syndrome that mimics central nervous system infections, making diagnosis difficult and delaying treatment.