Ameloblastic carcinoma is a rare odontogenic tumour of the jaws, the most commonly involved area is the posterior portion of the mandible, occurs in the mean age of 30.1 years and there is no apparent sex predilection. The most common sign described has been swelling, include associated pain, rapid growth, trismus and dysphonia. There are some different in the terminology of ”Malignant ameloblastoma” and ”Ameloblastic carcinoma”; ”Malignant ameloblastoma” refers to a neoplasm in which typical histologic features of ameloblastoma are seen in the primary tumour located in the jaw as well as in any associated metastatic deposits, and ”Ameloblastic carcinoma” described as ameloblastomas in which there had been histologic malignant transformation. The treatment of ameloblastic carcinoma has been recommended that surgical treatment with 2-3cm bony margins of jaw resection, the effects of radiotherapy and chemotherapy are as yet unproven. We reveal a case of ameloblastic carcinoma of the mandible and report present illness, clinical exam, surgical therapy treatment and post-operation follow up in particulars.