Esthesioneuroblastoma is a rare tumour arising from the olfactory epithelium of the nasal vault which frequently invades the cranial base and orbit. Esthesioneuroblastoma has a bimodal age distribution between 11 and 20 years and between 51 and 60 years. Esthesioneuroblastoma accounts for approximately 1-5% of intranasal cancers. The case is reported of a 79-year-old female patient with a Kadish stage C tumour with a one-year history of headache, nasal obstruction, anosmia, rhinorrhoea and epistaxis. Aim of this study is to analyse the natural history, treatment and prognosis of this tumour, based on a review of the literature.