Central granular cell odontogenic tumor (CGCOT) is a rare benign odontogenic neoplasm reported with various terms and is not included in the 2005 WHO classification of odontogenic tumors. It shows a predilection for middle-aged women, usually presenting as an asymptomatic swelling of the mandibular premolar-molar region. Radiographic manifestations include unilocular or multilocular radiolucent lesion, but mixed density lesions can also occur. Histopathology shows sheets and lobules of round to polygonal cells with abundant eosinophilic, granular cytoplasm and eccentrically placed nuclei. Immunohistochemical and ultrastructural features suggest the histiocytic origin of granular cells. The neoplasm does not exhibit an aggressive biological behavior and hence enucleation or curettage ensuring complete removal remains the treatment of choice. We present a systematic review on clinical, radiological, histopathological, immunohistochemical and treatment aspect of CGCOT. Published articles were identified through a literature search using online databases (PubMed, MED-LINE, Scopus) and cross-references for papers published from the year 1950 to March 2013. From a total of 37 papers, 26 (38 cases) were extracted from the database for systematic review.