Esthesioneuroblastoma is an uncommon malignant neoplasm of the nasal vault that in the past was considered benign or low-grade malignant. Surgical approaches in the main were transnasal, with a high recurrence rate and ultimate patient death. With the modern imaging of CT and MRI, should the patient be willing and fit enough, esthesioneuroblastoma currently should be approached using a craniofacial resection. Large tumors should be considered for preoperative chemotherapy and postoperative radiotherapy. Local tumor recurrence is not uncommon and is generally related to the attention to local anatomic dissection. Neck metastases, when they present, should be excised using a modified neck dissection. Distant metastases may present at any time during the course of the disease, generally within 36 months, and may respond to local radiotherapy or systemic chemotherapy. Five-year survival currently appears to be optimized by surgery followed by postoperative radiotherapy and is approximately 65%.