Esthesioneuroblatoma (ENB) is a rare tumor arising from the olfactory epithelium of the nasal vault which frequently invades the cranial base, cranial vault and orbit. ENB has a bimodal age distribution between 11 and 20 years and between 51 and 60 years. ENB accounts for approximately 1 to 5% of intranasal cancers and no consensus has been reached regarding treatment of this tumor. We report on a 66 year old female patient with a Kadish stage C tumor with frontal lobe invasion submitted a total craniofacial resection with a combined head neck and neurosurgeon team. The purpose of this study is to analyze the natural history, treatment and prognosis of this tumor, based on the literature review.