Ameloblastoma is a rare benign odontogenic epithelial tumor characterized by abnormal cell growth, which easily infiltrates and destroys surrounding bony tissues. Clinically, it is mostly seen in the ascending ramus area, in men in their fourth and fifth decades more frequently than in women. One of the 3 clinical variants of ameloblastoma is desmoplastic type, with involvement of the maxillary arch more often than the mandible. Its histopathologic variant characterized by extensive squamous metaplasia, islands of tumor cells, and sometimes keratin formation is known as desmoplastic acanthomatous ameloblastoma.The aim of this report was to present an unusual case of symphysis located desmoplastic acanthomatous ameloblastoma in a 56-year-old female patient, who was experiencing laryngeal carcinoma 2 years ago.