Esthesioneuroblastoma is a rare tumor of the olfactory epithelium. This report analyzed 11 children and adolescents treated in a single institution between 1982 and 2002. For 9 patients, therapy consisted of an initial course of chemotherapy before surgical resection and postoperative radiotherapy, for 1 patient an initial course of chemotherapy before radiotherapy and for another resection before radiotherapy with no chemotherapy. Response to chemotherapy was assessed in 9 patients of whom 6 achieved a complete or a partial remission. Ten patients are long-term survivors. The 5-year actuarial disease-free survival and overall survival rate was 91% (95% confidence interval, 62%-98%). Our study indicates that esthesioneuroblastoma is sensitive to chemotherapy and supports the role of combined modalities including neoadjuvant chemotherapy, surgery, and radiation therapy.