Behçet's disease is rare in childhood. We describe a 10-year-old boy with neuro-Behçet's disease (NB) who presented with fever, headache, vertigo, and hearing loss. An examination of the cerebrospinal fluid (CSF) revealed pleocytosis as well as elevated protein and interleukin (IL)-6 levels. Brain magnetic resonance imaging (MRI) showed hyperintensity of the right thalamus and midbrain on T2-WI, and gadolinium (Gd) enhancement of left acoustic nerve origin. HLA-B51 was positive. Prednisolone combined with methotrexate resulted in a complete remission. Brain MRI and the CSF IL-6 level were useful for the diagnosis and monitoring of this pediatric patient with NB.