Malignancies of the nasal cavity are uncommon and comprise less than 1% of all malignancies. While almost half of the sinonasal cancers are squamous cell carcinomas, olfactory neuroblastoma, also known as esthesioneuroblastoma, comprise less than 6% of nasal malignancies. After the initial description in 1924 by Berger and Luc, less than 1,000 cases were reported until 1997. These are tumours of neuroectodermal origin which are thought to arise from the olfactory epithelium in the nasal cavity. The mean age at presentation is 53 years, with most cases occurring in patients between 40 and 70 years of age. Although there is a male predominance, no other risk factor is commonly associated with this disease.