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