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Objective: Pediatric esthesioneuroblastoma (ENB) is a rare entity with only one previous report of an ectopic origin. Much of the research available regarding ENB is in the adult literature, with few studies focusing on the pediatric population. Here we report a case of ectopic pediatric ENB and review the literature regarding this unusual clinical entity. Methods: Case report (n = 1) describing presentation, operative findings, and clinical course followed by a literature review of ENB with a special focus on pediatric cases, including ectopic origins. Results: A 13-year-old boy presented with a unilateral nasal polyp without history of epistaxis. The patient underwent endoscopic resection with pathology revealing ENB. There was no tumor at the olfactory groove and was found to arise from the lateral nasal wall. The patient had postoperative proton beam therapy and no evidence of disease at his six-month follow up. Pediatric ENB is rare and often presents at a much more advanced stage than adult ENB. Due to the paucity of cases, its management follows that of conventional ENB including multimodality therapy with surgery and radiotherapy. Recurrences have been reported beyond five years, requiring long term follow up. Conclusions: Pediatric ectopic ENB is a rare clinical entity that requires a high index of suspicion for diagnosis. Multimodality therapy with surgery and radiotherapy is the standard of care, and patients should be counseled on long term follow up.