Cephalocele involving the oral cavity

A cephalocele is an uncommon neural tube defect that rarely involves the oral cavity. We present a case of an oral cephalocele associated with polyhydramnios discovered on antenatal ultrasound. The differential diagnosis includes epignathus and epulis. The prognosis is dependent not only on the extent of brain involvement and associated anomalies, but also on the exact location of the herniated mass. If the oral cavity is extensively involved, airway management at birth may be difficult. Therefore, preparations for delivery may include maternal tertiary site referral, antenatal neurosurgical and pediatric consultation, and anticipation of complications such as rupture of the sac, dystocia, or the need for immediate tracheostomy if intubation is not possible.