Ameloblastomas are uncommon tumours arising from odontogenic epithelium and represent only ~1% of all tumours and cysts in the jaws. Although they exhibit locally invasive behaviour, they are classified as benign tumours. In a very small subset of cases, ameloblastomas may undergo malignant transformation. At present, the current WHO classification stratifies these malignancies into three distinct entities: metastasising (malignant) ameloblastoma, ameloblastic carcinoma and ameloblastic fibrosarcoma.

In this presentation, we present a case series to illustrate the malignant variants of ameloblastoma: two cases of metastasising ameloblastoma, and one extremely rare case of a mixed odontogenic tumour comprising of carcinoma ex-ameloblastoma and odontogenic carcinosarcoma. The latter disease entity is not in the current WHO classification.

 The small number of these malignant pathologies present a management challenge owing to the lack of established treatment protocols. Hence, we present a literature review on available data to discuss its presentation, pathology, treatment and patient outcomes.