The most common cause of difficult intubation in pediatrics is due to congenital anomalies of airway. We report a case of neonate with congenital epulis (CE) who presented with a difficult airway. A 7-day old neonate weighted 3.2 kg with a large mass occupying the oral cavity that was diagnosed as congenital epulis was scheduled for excision biopsy. The mass was large, mobile, and moving in and out with no clear pedicle. An intravenous line was inserted and secured. The airway was then assessed while the patient was awake and an assistant displaced the mass and a laryngoscope was placed to visualize the larynx easily. After preoxygenation, inhalation induction of anesthesia was accomplished using sevoflurane in oxygen. Endotracheal intubation was performed with conventional laryngoscopy. The baby made uneventful recovery after the surgical procedure. In conclusion, epulis presents a real challenge to anesthesiologists. It can be excised either under local or general anesthesia, depending on the size of its pedicle. If done under general anesthesia, assessment of the airway is mandatory for better airway management and safe endotracheal intubation.