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Epidermal inclusion cyst (EIC) is rare in head and neck region with incidence in oral cavity being less than 0.01%, presenting as congenital or as acquired lesions. Clinically patient presents with dysphagia, difficulty in breathing and may be associated with various syndromes like Gardner syndrome, basal cell nevus syndrome and pachyonychia congenita. Pathogenesis of EIC includes implantation of ectodermal component along the embryological site of fusion or due to trauma. A case of EIC of tongue in a three year old male child is presented here along with review of literature.