Ameloblastic carcinoma is a rare and aggressive malignant odontogenic tumour that can arise de novo or from a preexisting benign lesion. It most frequently involves the mandible, and its clinical course is aggressive with extensive local destruction. Although rare, these lesions have been known to metastasize, mostly to regional lymph nodes or lungs. Surgical therapy, eventually followed by radiotherapy, is the treatment modality most frequently used, while the role of chemotherapy remains unclear. Here we present a case of secondary ameloblastic carcinoma of the mandible in a 33-year-old male patient with typical aggressiveness and extensive local destruction and metastasis with a follow-up period of 93 months.