Cases of Castleman's disease, a disorder affecting lymphoid organs, which is largely benign in nature, are rare in the pediatric period. This report describes one such case, occurring in a 5-year-old boy, and reviews the published cases of Castleman's disease presenting in childhood and adolescence. The purpose of this review was to compare the features of this disease process in children with the much fuller documentation that exists for Castleman's disease in adults. In children, Castleman's disease usually presents in the abdomen, chest (mediastinum or lung hilum), or neck and may be either asymptomatic or present with systemic symptoms of which anemia and fever are the most frequent. The disease is curable by surgical excision.