An 8-year-old boy presented with a mass located in the portal hilum and hepatosplenomegaly, and the presumed initial diagnosis was lymphoma. The pathology result was Castleman disease of hyaline vascular type. Castleman disease is an unusual diagnosis that should be kept in mind in the differential diagnosis of portal masses. In the case of solitary lesions, total excision can be curative. (C) 2011 Elsevier Inc. All rights reserved.