Olfactory neuroblastoma is a rare sinonasal malignant tumor which often presents with non-specific clinical symptoms as nasal obstruction, epistaxis, or anosmia. The diagnosis of olfactory neuroblastoma requires pathologic examination. In 2013, we encountered a case of olfactory neuroblastoma classified as Kadish stage group C, and T4N0M0 stage IV. After lengthy discussion, the patient underwent transnasal endoscopic biopsy and concurrent chemoradiotherapy. The post-treatment MRI showed shrinkage of the tumor. Follow-up MRI at 6 months and 1 year after treatment showed no evidence of tumor recurrence. As olfactory neuroblastoma is a relatively uncommon disease, we reported this case and discussed the pathological staging, clinical staging and treatment modality.