The aim of this study was to evaluate neurological involvement in a series of 110 North Italian patients with Behçet disease (BD), a multisystemic vasculitis of unknown origin, followed up for a period of 5 years. During this time, 27 (24.5%) patients with neuro-BD were identified. Twenty out of 27 showed at least one acute attack in their clinical course. In 14 of them, a neurological evaluation was carried out during the attack. The other 13 patients were evaluated during a remission phase. The onset of neuro-BD was usually characterized by an acute attack with motor symptoms (66.6%) and behavioural/cognitive changes (47.6%), while headache was more frequent in the remission phase (76.9%). On magnetic resonance imaging, large brain-stem/diencephalon lesions were usually seen during the attack. In the remission phase, they were often located in the white-matter. Aspecific cerebrospinal fluid abnormalities were usually seen during the attacks. Cerebrospinal fluid analysis together with radiological and clinical features seems to be useful for the differential diagnosis in these patients.