Central odontogenic fibroma (COF) is a rare neoplasm of the jaw. The combination of COF and Central Giant Cell Granuloma (CGCG) is exceedingly rare. To the best of our knowledge just 57 cases of hybrid COF-CGCG have been reported worldwide. Here we report a case of hybrid COF-CGCG in a 31-year-old woman. a well-defined radiolucency in the premolar region of the mandible was seen. Surgical excision was performed, and histologic study shows fibro-cellular tissue containing giant cells, zones of mesenchymal cells proliferation, in a whorled pattern and, multiple cords and small nests of odontogenic epithelium. Also, this report presents a literature review of previously reported cases.