The epidermoid cyst is a frequent dermatological entity, being very rare its location in the oral cavity, with a higher prevalence at the level of the floor of the oral cavity. It is considered of congenital etiology, although it occurs predominantly in young adults. They are generally well circumscribed, slow growing and asymptomatic masses, although those located in the oral cavity and oropharynx can cause phonatory, swallowing and respiratory disorders. The diagnosis is clinical, being the differential diagnosis with the dermoid and teratoid cysts only possible with the histopathological study. The treatment is surgical and the prognosis is favorable without relapses. We present a case of epidermoid cyst located in uvula, in a male of 6 months of life, treated surgically, with the tumor excision by a transoral approach