Introductıon: Osseous choristoma is a benign lesion that is characterized by proliferation of mature osseous tissue in an abnormal anatomical location. The lesion, which was first described by Monserrat in 1913 as “lingual osteoma” has been called “osseous choristoma” since 1971. Here, we present a rare case of osseous choristoma located on the tongue and a review of the literature.Case Report: A 26 year-old male patient was referred to the Department of ENT with the complaint of swelling of the tongue. Physical examination showed a nodular, sessile lesion on the base of the tongue and the lesion was removed. Histopathological examination of the material revealed a well-defined, submucosal lesion consisting of mature bone tissue and was reported as “osseous choristoma”.Conclusıon: Osseous choristoma is a benign lesion that occurs mostly in women in their second or third decades of life, although it can be seen in a wide age range. Osseous choristoma may occur in different locations of the oral cavity and maxillofacial region such as the tongue, buccal mucosa, alveolar mucosa, submandibular region, submental region, masseter muscle and palate. This rare entity should be kept in mind because it may be confused with other benign lesions of the tongue like hemangioma, lymphangioma, hamartoma and malignant tumors.