Cartilaginous choristoma of the tongue is a rare lesion. We report a case of cartilaginous choristomaarising from the margin of the tongue, with a review of the literature. A 16-year-old girl was referred to our department because of a small asymptomatic mass on the posterior margin of the tongue of 5 year’s duration. The lesion was approximately 3 mm in diameter and pediculate. The lesion was excised under general anesthesia, and the pathological diagnosis was cartilaginous choristoma. Histopathological examination of the excised specimen revealed a submucous spherical nodule composed of heterotopic hyaline cartilage, myxoid fibrous tissue, and circumscribed fibrous connective tissue. A gradual transition was noticed among these tissues. The histopathological findings suggested that the metaplastic formation of cartilage was caused by the transformation of fibrous tissue into cartilage.