Introduction Olfactory neuroblastoma originates from the neuroectoderm of the olfactory nerve and accounts for 3% of nasal tumors. It has been named as olfactory neuroepithelioma, olfactory neuroblastoma, and sensory neuroblastoma because its exact tissue origin is not clear. In 1993, the WHO Pathologic Classification of Tumors classified olfactory neuroblastoma as a neuronal tumor, and included olfactory neuroepithelioma in parentheses. The disease can be seen at any age, with a double peak at ages l0-20 and 50-60, and is more common in males. The tumor usually occurs in the top, upper and lateral walls of the nasal cavity, with a slow progression and locally aggressive growth. In the early stage, the main symptoms include nasal congestion, rhinorrhea, headache, runny nose, loss of smell and other symptoms of chronic sinusitis, with the highest rate of loss of smell. In the progressive stage, patients may also develop ocular symptoms such as tearing, proptosis, diplopia and visual impairment. Very few patients have neuropsychiatric symptoms such as convulsions and loss of consciousness. The tumors in the upper part of the nasal cavity are grayish-red, brittle, and bleed easily when touched. At present, the clinical staging of this disease is mostly proposed by Kadish et al: stage A tumor is confined to the nasal cavity; stage B tumor invades the sinuses; stage C tumor is beyond the nasal cavity and sinuses and may invade the sieve plate, orbit, skull base, neck or distant metastasis. CT can well indicate the damage of tumor tissue to its surrounding bone structure, while MRI can clearly show the extent of infiltration of tumor tissue into adjacent soft tissues, especially intracranial and orbital areas. The diagnosis and differential diagnosis of ENB is insidious and the clinical manifestations are non-specific. The diagnosis depends mainly on pathological examination. The tumor needs to be differentiated from undifferentiated carcinoma, malignant lymphoma, and embryonal rhabdomyosarcoma under low magnification.