Neuro-Behçet's disease (NB) is classified into acute and chronic progressive types. Acute NB occurs as acute meningoencephalitis with focal lesions, presenting with high-intensity areas on T2-weighted images or flat images on magnetic resonance imaging (MRI). Chronic progressive NB is characterized by intractable, slowly progressive dementia and ataxia with progressive brain stem atrophy on MRI. The most important feature of chronic progressive NB is persistent elevation of cerebrospinal fluid (CSF) IL-6 levels. The progression rate of the brain stem atrophy has been shown to be closely correlated with integrated CSF IL-6. For the correct diagnosis of acute or chronic progressive NB, it is mandatory to confirm the diagnosis of Behçet's disease. Regarding treatment, all of corticosteroids, cyclophosphamide, and azathioprine are ineffective to treat chronic progressive NB. Low-dose methotrexate (MTX) is effective in decreasing CSF IL-6 levels without promoting the progression of neuropsychological manifestations. Infliximab has a beneficial effect in patients with inadequate response to MTX. Recently, clinical guidelines were established for the management of acute NB and chronic progressive NB, which is helpful for routine clinical practice.