Abstract :Congenital granular cell tumour or congenital epulis is an uncommon benign tumor of the oral cavity of newborns. The condition may interfere with respiration, feeding or lip closure. Antenatal ultrasound diagnosis is possible and it can avoid unexpected situations. Surgical excision is the first line treatment but, occasionally, the lesion may present spontaneously regress postpartum. The aim of this case report is to describe the clinical presentation and treatment of the congenital granular cell tumour in the maxillary arch of a 2-hour-old female newborn.