Esthesioneuroblastoma (ENB), also called olfactory neuroblastoma, is a cancerous tumor originating from the olfactory neuroepithelial cells frequently invading the brain through the cribriform plate. The optimal therapy is the multimodality treatment involving a group of physicians trained in different medical specialties. Establishing a careful histopathological diagnostic and treatment planning based on a multidisciplinary approach is of paramount importance. The treatment of ENB correlates with the extent of the lesion, with surgery being the mainstay of therapy followed by postoperative irradiation. Surgery, when complete, image-verified and associated with radiation therapy results in long-term survival and presents a very low probability of illness recurrence. We present the case of a 46-year-old female with ENB, who was operated on in the Clinic of Neurosurgery of the National Institute of Neurology and Neurovascular Diseases in Bucharest, Romania, through a bifrontal craniotomy approach. Gross total resection of the intracranial extent was performed. The pathological diagnosis revealed an aggressive olfactory neuroblastoma. Three weeks after discharge from hospital, the tumor was completely resected through a lateral rhinotomy performed by an otorhinolaryngologist. Six weeks later, the patient received adjuvant therapy (radiotherapy and chemotherapy). The outcome was favorable, with no tumor recurrence at 20 months postoperatively. Our case demonstrates that even when dealing with a visibly aggressive tumor, a correct diagnosis, accurate classification and grading along with appropriate therapy ensure a favorable outcome.