We report a case of Behçet's syndrome with myositis in a pediatric patient, emphasizing the importance of muscle involvement in the differential diagnosis of calf pain and swelling in Behçet's syndrome. A review of the English-language literature from 1965 to the present suggests that the clinical picture of Behçet's syndrome in children differs from that in adults, in that there is a lower frequency of ocular disease, and unusual manifestations appear to be more common.