We report an extremely rare case and localization of polypoid angiomiofibroblastoma tumour, a case report and review of the world literature concerning angiomiofibroblastoma tumour. We present the case of a 74-year-old man who underwent left anterior and posterior ethmoidectomy with extirpation of tumour mass from left nasal cavity, epipharinx and left sphenoid sinus. The prognosis for this group of tumour is good and patient didn't receive any kind of therapy except surgical treatment. To our knowledge, this is a rare report in the world literature of polypoid angiomiofibroblastoma tumour of nasal cavity. This case indicates that angiomiofibroblastoma tumour of nasal cavity and paranasal sinuses is a rare disease including its localisation which otolaryngologists should be aware of and one which should be included in the differential diagnosis of tumours involving sinonasal tract.
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