Background: Epidermoid or epidermal inclusion cysts are a rare cause of swelling in oral cavity. Case Report: This is the youngest and to best of our knowledge the largest reported case of congenital epidermoid cyst of the tongue. Excision of cyst was planned as patient was not able to close the mouth. Histopathological assessment of patient labeled the lesion as epidermoid cyst. Discussion: High index of suspicion must be maintained for epidermoid cyst in oral swellings under the tongue.