Ameloblastic carcinoma (AC) is a malignant form of ameloblastoma and an extremely rare odontogenic tumor.Herein, we report a case of AC of the maxilla. A 62-year-old woman presented to a dental clinic with tenderness of the right cheek. A panoramic radiograph showed a radiolucency in the right maxilla, and she was referred to another hospital. With a provisional diagnosis of a benign lesion, she underwent surgical removal of the maxillary mass, following which a pathological diagnosis of AC was made. Subsequently, she was referred to our department for further treatment, and she underwent partial maxillectomy. She remained disease-free at 3-year follow-up. We performed a literature review of AC cases reported in the last decade. Of 36 AC cases, 63.9% were clinically diagnosed as benign lesions ; among them, 52.2% cases were clinically diagnosed as ameloblastoma. Conversely, 92.6% cases were diagnosed as malignant lesions on biopsy ; among them, 76% cases were pathologically diagnosed as AC. These findings suggest that clinical and imaging findings may complicate the differentiation of AC from ameloblastoma ; however, performing a biopsy can help obtain a correct diagnosis, leading to appropriate treatment planning.