Neuro-Behçet’s disease (NBD) is one of the most serious manifestations of Behçet’s disease (BD) that causes long-term morbidity and mortality, seen in 3 to 9% of the patients. It is rather infrequent in the pediatric age group. The most common neurological complaint in BD is headache; however, mostly it represents primary headache disorders rather than neurological involvement. Neurological manifestation of BD is almost always limited to CNS; presented in two main patterns. The most common form is parenchymal involvement affecting the mesodiencephalic region and brainstem, and non-parenchymal involvement occuring mostly in the form of cerebral venous sinus thrombosis (CVST). Treatment of NBD mainly consists of high-dose corticosteroids at the acute stage, which should be tapered over a few months, and immunosuppressive agents for long-term prevention of relapses. Drugs such as azathioprine, mycophenolate mofetil, cyclophosphamide, and anti-TNF medications are commonly used as long-term treatment; cyclosporine should be avoided. Neurological involvement is a severe and feared manifestation in patients with BD; however, with early diagnosis and early effective treatment options, it can be controlled without further damage. Over-diagnosis is an important risk in patients with suspected NBD; in such cases, expert opinion should be sought.