Two childhood with Behcet's disease were reported. To evaluate the clinical features of Behcet's disease in childhood, fifty eight case studies (domestic; 19 cases, world-wide; 39 cases) were examined from the literature including two cases in our clinics. Oral ulcers (100%), genital ulcers (82.8%), skin manifestations (77.6%), and articular involvement (51.7%) were frequently found as seen in adult cases. Gastrointestinal manifestations were more common in childhood than in adults (50.0% vs. 29.3%). However, ocular inflammatory disease was less frequently seen in pediatric patients as opposed to adults. Three fourth of children with Behçet's disease was effectively treated with oral or intravenous prednisolone. These findings suggested that the criteria for childhood Behçet's disease will be necessary for early and proper diagnosis.