Esthesioneuroblastoma, a malignant neoplasm arising from olfactory epithelium, is unusual in the pediatric age-group. Management has traditionally involved surgery and radiotherapy, alone or in combination, with chemotherapy reserved for recurrent or high grade disease. We report a single institution experience utilizing chemotherapy and radiotherapy as the initial treatment and successful control of the primary tumor in two patients. In one patient, neck dissection and high dose chemotherapy combined with autologous bone marrow transplantation were used as successful salvage therapy of neck metastasis. Both patients are alive and disease free with a mean follow-up of 56 months. These results support the role of chemotherapy in the treatment of esthesioneuroblastoma and suggest that chemotherapy be used as part of the initial combined modality treatment plan.