Juvenile ossifying fibroma is a rare benign neoplasm with locally aggressive growth and high recurrent rate. Juvenile ossifying fibroma is classified into juvenile psammomatoid and trabecular type by histological findings. juvenile psammomatoid ossifying fibroma is generally seen in younger age group and the most common site is paranasal sinuses, orbits, and fronto-ethmoidal complex. Clinical symptoms vary from diplopia, nasal obstruction and headache to cosmetic disfigurement, depending on the location and the size of lesion. Complete excision is the treatment of choice because of its high tendency to recur. The author recently experienced a psammomatoid ossifying fibroma of anterior skull base in a 16-year-old boy who had the past history for about completely healed acute lymphocytic leukemia. We removed that tumor by endonasal endoscopic transseptal approach. We present a case of JPOF, and a comprehensive review on its clinical, histo-pathological, therapeutic and prognostic aspects.
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