OBJECTIVE Neurologic involvement in the pediatric population with Behçet disease (BD) is limited to case reports. The aim of this study is to examine the frequency and type of neurologic involvement in pediatric patients with BD. METHODS Medical records of 728 patients with a diagnosis of neuro-BD (NBD) of 2 large BD cohorts followed in Istanbul University were included in the study. Patients with an onset of both systemic and neurologic symptoms at or before age 16 (pediatric neuro-BD) were identified. Demographic and clinical characteristics of pediatric patients with NBD were compared with adult patients with NBD. RESULTS There were 26 cases with pediatric BD (3.6%) and 702 (96.4%) adult-onset patients. Gender ratio was equal in the general pediatric BD cohort, whereas male/female ratio was 5.5/1 in pediatric NBD cases. Mean age at BD onset and neurologic involvement onset were 13.0 ± 3.0 and 13.5 ± 2.4, respectively, and in the adult population mean age at onset of BD was 26.7 ± 8.0 and neurologic involvement occurred a mean of 5.3 ± 4.5 years later. Clinical and MRI evaluation revealed that 3 children had CNS parenchymal involvement and 23 had dural venous sinus thrombosis (88.5%). We observed parenchymal involvement in 74.8% of the adults, contrary to the low 17.2% of cases with venous sinus thrombosis. CONCLUSIONS Pediatric NBD comprises 3.6% of our whole NBD cohort, with a male predominance, mainly in the form of dural venous sinus thrombosis, whereas in the adult NBD population the dominant form of neurologic involvement is parenchymal, suggesting that the pathogenesis of NBD may be different according to the age at disease onset.