Osteogenic sarcoma of the Craniofacial region are uncommon.They have been mainly reported in the mandible and the maxilla, Primary osteosarcoma arising from ethmoid sinus is an extremely rare condition. We report a case of 34 year old male, presented with gradually increasing swelling over the right medial canthus with history of double vision for one year. Clinically we thought it was a mucous retention cyst in the ethmoid area. After doing Contrast enhanced CT, we proceeded with transnasal endoscopic removal of the cyst. Histopathological examination of the specimen surprisingly turned out to be a low grade Osteogenic sarcoma. Patient was treated with adjuvant chemotherapy.
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