The central granular cell odontogenic tumor (CGCOT) is a rare, benign, slowly growing, odontogenic neoplasm. CGCOT was not considered as a distinct entity in the WHO classification reported on 2017. This study reports a rare case of CGCOT involving the right side of maxillary anterior region of a 39-year-old white woman. In addition, to better delineate the clinical, radiographic, histopathologic and immunohistochemical characteristics of CGCOT, a literature review of all published cases (in PubMed/ Google Scholar/ MEDLINE/Scopus) of CGCOT is provided. CGCOT is a very uncommon tumor, with only 51 reported cases in the literature. The present case is interesting regarding to its rarity for being in the maxillary anterior region, which has not been previously reported in Asia. The immunohistochemical findings of the current case and other cases in the literature review, verified the mesenchymal origin of granular cells and odontogenic nature of the epithelium islands, which can be a possible promise for placing this lesion in the future WHO odontogenic tumor classification.