Nasopharyngeal carcinoma is a tumor originating from the surface epithelial cells of nasopharynx. It is rare in children and adolescents. Most common physical finding is a neck mass. Most children with nasopharyngeal carcinoma present with advanced stage disease. The presentation with hematological abnormalities in patients without systemic metastasis is extremely rare. We reported a 14-year-old boy presenting with a mass at the right side of the pharynx and leukemoid reaction. To our knowledge, this is the first report of leukemoid reaction associated with pediatric nasopharyngeal carcinoma in English literature. (C) 2014 Elsevier Ireland Ltd. All rights reserved.