ObjectiveEsthesioneuroblastoma (ENB) is a rare tumor of the olfactory epithelium. The objective of this study was to evaluate treatment modalities including surgery, IMRT, and chemotherapy and patient outcomes.Patients and MethodsA retrospective analysis was performed on a total of 21 patients. Therapy included craniofacial resection (CFR), radiotherapy, chemotherapy, or a combination of these methods.ResultsThe median follow‐up period was 47 months. Surgery was performed in 90.4% of cases; radiotherapy was performed adjuvantly in 15 (72.7%) patients. Surgery, radiotherapy, and chemotherapy were administered to 7 (33.3%) patients. Eight (38.3%) patients had local recurrence. The 5‐year crude overall survival was 71.4% and actuarial 5‐year overall survival was 58% with confidence interval (CI, 25 and 81, respectively). The 5‐year crude disease‐free survival rate was 59% and the 5‐year actuarial disease‐free survival rate was 62% (CI, 28 and 83, respectively).ConclusionMultidisciplinary therapy of ENB should be considered, especially for Kadish C and high‐grade lesions. Craniofacial resection (CFR), Intensity modulated radiation therapy (IMRT), and chemotherapy should be investigated in a multi‐institution trial of ENB.© 2007 American Academy of Otolaryngology‐Head and Neck Surgery Foundation. All rights reserved.