We present a case of a female neonate, born at 36 weeks of gestation from a monochorionic, diamniotic spontaneous twin pregnancy with congenital epulis, 2.5 cm in size, protruding from the oral cavity. Histopathology revealed a typical granular cell lesion. The other twin, also female, was prenatally diagnosed with congenital heart defect: pulmonary stenosis. The tumor was typically located in the maxillary alveolar ridge and unidirectional. The child underwent a successful surgery on the first day after birth. The course of the procedure and recovery was uneventful.