Esthesioneuroblastoma is an uncommon tumor that presents in the sinonasal cavity and anterior skull base. Cervical metastases are not frequently found on initial presentation but eventually occur in 20-25% of these patients. This presents the treating physician with the difficult decision as to how and when to treat the neck in this disease. The aims of this study were to provide a comprehensive review of the incidence of N+ disease at presentation, make recommendations about the optimal treatment strategy of patients with N+ disease, explain the role of elective neck treatment in patients with N0 disease, and comment on treatment of patients with late cervical metastases that require salvage therapy, using the literature review of the incidence and treatment of neck disease in patients with esthesioneuroblastoma. This review revealed an approximately 5-8% incidence of cervical nodal metastasis at the time of presentation. Combined modality therapy with surgery and radiotherapy is recommended to treat the N+ neck at the time of diagnosis and later. Chemotherapy may have a role combined with radiation treatment, but there are little data to support this. There is limited evidence to substantiate the use of elective neck dissection or elective radiotherapy in the clinically and radiologically N0 neck. Patients who have late cervical metastases have a clear survival advantage (59 vs. 14%) when treated with combined surgery and radiotherapy relative to single modality methods alone. The results indicate that the management of the neck in esthesioneuroblastoma continues to be a significant challenge in the treatment algorithm of these complex patients.