Congenital fusion of mandible to maxilla (syngnathia) is rare. Only 25 cases of syngnathia have been reported up to date since the first case was reported in 1936. Of these cases only six have involved fusion of the ascending rami of mandible to the maxilla and zygomatic complex. This report is an additional case of syngnathia involving bilateral bony fusion of mandibular horizontal and ascending rami to zygomatic complex and maxilla in a female neonate. Delay in treatment afforded the opportunity to observe the effects on the growth of the patient as she presented again at the age of 3 years.