Congenital epulis, known as a congenital gingival granular cell tumor, is a benign tumor and very rare in newborns. Voluminous or multiple tumors can cause mechanical obstruction of the oral cavity and may result in postnatal feeding and respiratory problems.

We report the clinical case of a female full-term newborn who presented a tumor on the upper gum obtruding into the oral cavity discovered at birth. The pregnancy was followed normally with three prenatal ultrasounds, which did not show abnormalities. The mass was excised under local anesthesia on the second day of life. The outcome was good after surgery and regular feedings were started on the second postoperative day. Histological examination confirmed the diagnosis of gingival tumor with granular cells and absence of signs of malignancy.

Prenatal diagnosis is fundamental in the therapeutic approach to this rare lesion but remains difficult because the findings are non specific and the generally late development of the tumor.