Objective: To describe the clinical, 18F-fluorodeoxyglucose (FDG) and 11C-choline PET/CT features of a case of esthesioneuroblastoma.Methods: In February 2010, an 18 year-old young man presented with the tinnitus of right ear and nasal obstruction.In July, he underwent a nasal enhanced computed tomography (CT) scan and an occupy lesion in the right parapharyngeal region was detected.The diagnosis of nasopharynx cancer was suspected.The nasopharyngo-fiberoscope was then performed and showed the swollen right lateral pharyngeal wall and shifting of the right Eustachian orifice.No obviously occupy lesion was found by the nasopharyngo-fiberoscope.Afterwards PET/CT was referred.18F-FDG PET/CT identified the occupy lesion in the right parapharyngeal region and showed the lesion was 18F-FDG negative.The patient was then scanned with 11C-choline PET/CT in the next day.11C-choline PET/CT demonstrated that the lesion had very intense uptake of 11C-choline.The disease was suggested to be a malignant lesion but not nasopharynx cancer by PET/CT.Results In order to avoid hemorrhea during the operation, blood vessel embolization was performed.Arteriography showed the tumor had an abundant blood supply and the blood supply was nearly obstructed after the embolization.Resection operation under the guide of endoscope was then performed.The lesion was completely resected and the diagnosis of esthesioneuroblastoma was established by pathohistology.Conclusions Utility of PET/CT in the detection of esthesioneuroblastoma was rarely reported.The present study signifies that 18F-FDG PET/CT has a potential possibility of false negative for esthesioneuroblastoma and 11C-choline PET/CT might be a supplement.