Esthesioneuroblastoma (ENB) or olfactive neuroblastoma is a rare cancer arising from the neuroepithelium of the olfactory epithelium of the nasal cavity. Sinusal, orbital and intracranial expansions are common. The anatomopathological diagnosis will frequently require immuno-histochemical tests and sometimes electron-microscopy as well as genetic testing. Medical imaging with CT scan and MRI is essential for the stadification. Treatment will generally associate debulking or curative surgery (with combined rhino-neurosurgical accesses) and conformal stereo-tactic radiotherapy. Chemotherapy was generally reserved for palliative cases; it can be now proposed as neoadjuvant treatment. Most patients will relapse; thus the follow-up will remain ad vitam.