Neonatal teratoma, a common congenital malformation, rarely occurs in the head and neck region, especially not within the oral cavity. This report presents a case of neonatal giant teratoma in the oral cavity and oropharynx along with cleft palate, which caused postnatal airway obstruction and respiratory distress and required postnatal resection in a female newborn. After the delivery and routine neonatal examination, the anesthesiologist conducted orotracheal intubation to establish the airway, and tumor resection was immediately done under local anesthesia. The optimal treatment of neonatal teratoma is exclusive emergent surgery. Immediate postnatal resection is necessary to prevent airway obstruction.