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Background: Central giant cell granuloma (CGCG), also known as giant cell reparative granuloma, is a non-neoplastic proliferative lesion with unknown etiology which commonly occurs in the right side of the mandible. This lesion presents a wide variety of radiological and clinical manifestations that may lead to misdiagnosis. In many situations, CGCG is diagnosed through histopathological examinations. Case: In this study, a case of CGCG with irregular clinical and radiological aspect arising from the anterior, left side of the maxilla was reported. Conclusion: In differential diagnosis of swellings arising from the anterior portion of the maxilla, the possibility of CGCG should be considered. Also, histopathological examinations are of great importance in the diagnosis of CGCG.