Ameloblastic carcinoma is a rare odontogenic malignancy that combines the histological features of ameloblastoma with cytological atypia, even in the absence of metastases. The major prognostic factor is the clinical course of the disease which includes its aggressiveness, local destruction, and distant metastatic spread preferentially through hematologic route if neglected. Histologically, ameloblastic carcinoma retains the features of ameloblastic differentiation and exhibits cytological features of malignancy in a primary or recurrent tumor. Because it is a very rare lesion, it poses a great difficulty in diagnosis. En bloc removal with 1–2 cm of normal bone margin has been regarded as the safest surgical modality to ensure disease-free survival. Literature search was carried out using the Boolean operator “And” between ameloblastoma and carcinoma on PubMed. Retrieved articles were extensively reviewed for epidemiology, etiology, diagnosis, treatment options, and prognosis of ameloblastic carcinoma.