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Fibrous dysplasia is a rare condition. We present a case of a 34-years-old man presented with left-sided facial pain, headache and nasal obstruction for a long duration. CT scan of the sinuses showed a mass occupying the left ethmoidal region abutting the orbit and skull base obstructing the left frontal sinus. The patient was treated successfully by image-guided endoscopic endonasal removal of the tumor with no recurrence after 3 years of follow-up. This report aims to show the usefulness of endoscopic endonasal removal of a large fibrous dysplasia of paranasal sinuses and skull base. [ABSTRACT FROM AUTHOR]