A case of epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma is reported. A newborn female presented with a large skin-covered mass arising from the palate, cleft palate, and lobulated tongue with a soybean-sized lingual lesion. The palatal and lingual masses were removed on the 9th day after birth. A cystic lesion was also found on the nasal septum after removal of the palatal mass; this lesion was resected at its base. Glossoplasty was performed after excision of the lingual lesion. Histologically, the palatal mass was diagnosed as epignathus. The cystic lesion was suspected to be meningothelial tissues on the basis of hematoxylin-eosin (HE) and immunohistochemical staining: Vimentin and epithelial membrane antigen were positive, and factor VIII was negative. The mass of the tongue dorsum was diagnosed as hamartoma. No recurrence of epignathus or evidence of meningocele have been observed after 5 years of follow up.