Epignathus or congenital nasopharyngeal teratoma occurring from intracranial space, growing out through complete cleft palate, and occupying oral and retropharyngeal cavities is an extremely rare abnormality. This unusual tumor may cause severe respiratory obstraction which is often associated with fetal prognosis in the newborn patients. Therefore, the antenatal diagnosis is essential for early management in the cases with congenital epignathus. Endotracheal intubation or tracheostomy should be caried out immediately after birth. We reported a case of nasopharyngeal teratoma which was diagnosed antenatally by ultrasonic echogram and amniography. Abnormal band between the tongue and the retropharygeal wall was excised by pediatric surgeons, enabling endotracheal intubation soon after birth in this case. Subsequently tracheostomy and gastrostomy were performed. The tumor has been excised successfully by three staged operations. She has been doing well taking food orally and breathing normally. CT and MRI was useful in delineating the tumor.
