It is also called as ³Hypognathous´. It is extremelyrare congenital defect characterized by absence of maxilla or mandible.
If mandible is absent the upper part of face may benormal and the skin of the lower part will becontinuous with the suprasternal integument.
The hyoid bone is sometimes absent, despite thepresence of a rudimentary tongue in the place of buccal orifice, there may be a vertical slit.
In the case of unilateral absence of mandibularramus, it is not unusual for ear to be deformed orabsent.