Deng L, et al. - Using the MeSH terms “ameloblastic” and “carcinoma,” a systematic review was conducted by searching PubMed and Medline databases to investigate the clinicopathological characteristics of ameloblastic carcinoma (AC) and review the literature to improve the diagnosis and treatment of the disease. One hundred twenty-five cases of AC from 81 eligible original studies and 18 cases of AC from this research have been included in the systematic analysis. According to results, 37 recurrence cases and 27 metastasis cases have been reported. The authors concluded that AC is a rare odontogenic epithelium neoplasm. A systematic review shows the need for early phase diagnoses and a close periodic assessment of recurrence and metastasis.