Congenital granular cell tumor (GCT) is a relatively rare intraoral benign tumor, approximately 200 cases of which have been reported in the neonatal period worldwide. The newborn infant may have feeding problems and respiratory difficulties due to airway obstruction. This lesion may be diagnosed by prenatal ultrasonography and simple resection is mostly required. We report a case of an adult type of GCT in a newly born infant, who presented with an intraoral protruding mass with a prenatal diagnosis. This article describes the prenatal course, clinical, and pathological characteristics, and management of the GCT.