Mucoceles of the lesser salivary glands are common benign lesions affecting all ages, most commonly appearing on the lower lip. However, mucoceles in neonates demonstrate a different clinicopathological pattern than in older children or adults and mandate urgent management. We present a case of a large mucocele on the tongue of a neonate, which impaired feeding and could block the airway. The lesion was resected on the 3rd day of life. We describe a surgical technique that facilitates this procedure. Our literature review revealed 13 neonatal lesser salivary gland mucocele cases, 7 of which were located on the tongue. All tongue cysts were large, impaired feeding and sometimes blocked the airway. Early treatment is usually imperative. Inclusion cysts outnumbered extravasation cysts. Prenatal diagnosis is important in order to arrange delivery in an organized center. Resection is the preferable procedure.