Prevalence of neurological involvement in Behçet's disease (BD) varies between studies and is associated with high morbimortality. Neurological involvement is classified as parenchymal and non-parenchymal involvement (vascular involvement). Treatment of neurological involvement consists of immunosuppressants. Evidence regarding presentation and outcomes in neuro-Behçet's (NB) is scarce. Observational retrospective study that included consecutive patients diagnosed with BD between 2006 and 2019 in a tertiary hospital of Madrid. Fifty-seven patients were included with a mean follow-up of 7.83 (±6.98) years. The mean age at diagnosis of BD and first NB episode was 34.7 (± 12.1) and 31.03 (±9.9) years, respectively. Fifty-six NB episodes were registered in 25 patients. Fever (OR 3.39, CI 95% 1.14-10.15) and pseudofolliculitis (OR 4.06, CI 95% 1.11-14.80) were associated to NB. Trigeminal neuralgia was found in 16% (4/25) of patients. The prevalence of NB in patients with BD was higher than previously described. Fever and pseudofolliculitis were more frequently associated with NB, with low mortality during follow-up.