INTRODUCTION: Many controversies exist in the diagnosis and management of this aggressively malignant condition, mainly because of limited literature and lack of randomized control trials, resulting in nonstandardized treatment methods. We performed a comprehensive review of the literature to identify management approach and treatment options for esthesioneuroblastoma. METHODS: An extensive review of the published literature was conducted in PubMed, OVID Medline, and EMBASE journals for studies of esthesioneuroblastoma. Terms for search included esthesioneuroblastoma (ENB) and olfactory neuroblastoma (ONB). No date restrictions were used. RESULTS: The search yielded 3876 related articles. Cross-checking of articles led to exclusion of duplicate articles. The remaining 1170 articles were screened for their full text and English language availability. Of 609 full-text articles available, animal studies, irrelevant articles, and studies with mixed/confusing data were excluded. We finalized 149 articles pertaining to the topic, including 119 original research articles, 3 book chapters, 11 reviews, 9 case reports, and 7 case series. CONCLUSIONS: Surgical resection followed by radiotherapy is the standard for treatment for higher-grade lesions. The endoscopic endonasal approach is gaining further recognition with more favorable outcomes and better survival than for open surgery. Postoperative radiotherapy is associated with the highest overall survival and shows benefit for patients with higher-stage disease and those who receive chemotherapy. Recurrence rates after treatment vary drastically in the literature and, therefore, prolonged follow-up with repeated imaging is recommended. Lifelong surveillance is recommended because of late recurrences associated with this tumor.