Nasopharyngeal teratoma is a rare benign entity, with most of the publ
ished literature represented by sporadic case reports. We present a re
cent case of a 23-year-old woman found to have elevated maternal serum
alpha-fetoprotein and ultrasound findings of a fetal cystic mass in t
he mandibular region. The infant had a large nasopharyngeal teratoma p
rotruding from the oral cavity making airway access extremely difficul
t. The presence of severe coexisting cardiac anomalies and respiratory
disease led to death in the neonatal period. This report and the revi
ew of the literature stress the importance of recognizing suggestive p
renatal data and preparing for perinatal management including maintena
nce of fetal oxygenation and aggressive early provision of an adequate
airway.