Intraoral osseous choristoma represents a benign lesion of growing ectopic bone in the soft tissues of the oral cavity. It is considered as rare entity while fewer than 100 cases have ever been reported worldwide. Nevertheless, the pathogenetic mechanism that drives this abnormal ossification still remains controversial. In the present study a case of lingual osseous choristoma in a 50-year old male is presented. The patient was treated with surgical excision and no recurrence was observed. In addition epidemiology, clinical presentation, and pathogenesis are reviewed, serving as a reminder of this rare pathology. Key words: Osseous choristoma, osteoma, tongue.