A case of enteric cyst in the anterior two-thirds of the tongue is reported. The patient, a 42 day-old female infant, presented with a protruding tongue mass since birth. Under the impression of ectopic thyroid gland or leiomyoma, complete resection was performed. The mass proved to be an enteric cyst, lined by small intestinal mucosa with an underlying coat of smooth muscle. Enteric cysts arising in the tongue is rare and only 8 such cases have been reported in the literature.