Ameloblastic carcinoma (AC) is a rare malignant epithelial proliferation that is associated with an ameloblastoma or histologically resembles an ameloblastoma. It is considered to be an aggressive neoplasm that is locally invasive and spread to regional lymph nodes or distant sites. It requires aggressive surgical treatment, and regular follow-up, therefore, differs from ameloblastoma. Sometimes, ameloblastomas exhibit a mild-to-moderate degree of cytological atypia; hence, in such cases, a correlation should be established between the clinical, radiological and histopathological findings, thus detecting the aggressiveness of the tumor. Here, we present the case report of a 52-year-old male patient diagnosed as AC based on histopathological and immunohistochemical findings.