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Ameloblastic carcinoma (AC) is a highly aggressive malignant odontogenic epithelial tumor. It may occur in a pre-existing ameloblastoma, odontogenic cyst, or de novo. The condition is typically painful, highly invasive, and multifocally metastasizing in nature, with the lungs being the most common secondary tumor site. The condition is most often encountered in adult men during the sixth decade, with a propensity towards the posterior mandible. Radiographic examinations typically exhibit ill-defined and aggressive lesions with massive bone loss. Histopathological examination shows classic cytonuclear atypia in the ameloblastic element. The immunohistochemical staining is generally found to be intense in markers such as Ki-67, p53, and p63. The majority of cases also carry mutations in the BRAF V600E gene. In this case report, we discuss the presentation pertaining to AC in a 29-year-old male patient, with an emphasis on the relevant clinical-radiological-pathological diagnostic aspects. The primary treatment of AC is surgical removal, occasionally in conjunction with dissection of the neck or radiation therapy to manage local and metastatic spread. In our case, surgical removal was performed. AC is a rare, aggressive, and invasive odontogenic epithelial malignant neoplasm. Further future studies regarding its pathogenesis, molecular genetics, and immunohistochemical aspects will help researchers in better understanding this novel entity.