Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare and aggressive type of cancer that originates in the nasal cavity. This uncommon malignancy arises from the olfactory neuroepithelium, the tissue responsible for the sense of smell. Esthesioneuroblastoma typically affects adults, with a higher incidence in the third to fifth decades of life. Symptoms of esthesioneuroblastoma can include nasal obstruction, epistaxis (nosebleeds), anosmia (loss of sense of smell), and facial pain or swelling. Due to its location, the tumor may also extend into nearby structures such as the sinuses, eyes, and brain, posing significant challenges for treatment. Diagnosis often involves imaging studies such as computed tomography (CT) scans and magnetic resonance imaging (MRI), as well as a biopsy to confirm the presence of esthesioneuroblastoma. The cancer is graded based on the Kadish system, which considers the extent of tumor involvement in the nasal cavity and surrounding areas. Treatment typically involves a combination of surgery, radiation therapy, and sometimes chemotherapy. The choice of treatment depends on the stage of the cancer, its location, and the overall health of the patient. Due to its rarity and complexity, a multidisciplinary approach involving specialists in otolaryngology, neurosurgery, and oncology is often necessary to develop a comprehensive treatment plan. Prognosis for esthesioneuroblastoma varies, with factors such as tumor grade, stage, and the success of treatment influencing outcomes. Despite its aggressive nature, early detection and prompt intervention can improve the chances of successful management. Regular follow-up and monitoring are essential for individuals who have undergone treatment for esthesioneuroblastoma to detect any recurrence or complications. Research into new treatment modalities and a better understanding of the underlying biology of this rare cancer are ongoing to improve outcomes for affected individuals.