Olfactory neuroblastoma (ONB), also known as esthesioneuroblastoma, is a rare malignant neuroectodermal tumor originating primarily from the basal layer of the olfactory epithelium in the roof of the nasal cavity. The most common symptoms are nasal obstruction, anosmia, recurrent epistaxis, rhinorrhea and excessive lacrimation. Imaging studies are helpful for assessing the extent of olfactory neuroblastoma, as well as grading the tumor. The definitive diagnosis is based on histopathological exam and immunohistochemistry profile and it might be quite challenging because the tumor can mimic other sinonasal malignancies. Because of the small number of reported cases and the lack of prospective research, the optimal treatment for olfactory neuroblastoma is still controversial. However, it is generally believed that surgical resection followed by radiotherapy gives the best outcome in terms of recurrence and survival rates. Keeping in mind the rarity of olfactory neuroblastoma and the diagnostic difficulty, the aim of this study is to improve the understanding of the clinical features by reviewing the literature focusing on the recent advances in the diagnosis and treatment of ONB. Moreover, we are reporting an atypical case of an aggressive type of olfactory neuroblastoma treated through a multimodal approach, with no signs of tumor recurrence after a 3-month follow-up.