Anencephaly is a congenital birth defect. While the
word anencephaly means « without a brain », it does
not accurately describe a child with such a defect.
Children with anencephaly do have a brain, but it is
not fully developed.
A baby with anencephaly is admittedly born with little
scalp, cranium vault, or brain, but he or she does
usually have part of the cerebral trunk. Your baby's
facial features will be complete; however, there will
be an opening in the skull. The size varies from child
to child. If you look into the opening, you will
see some brain tissue. Your baby's head can be
covered with a cap if you do not want to see this.
There may be other problems with your baby's body
(folded ears, cleft palate, open spine), but usually
your baby's body will develop normally.
(Source: http://www.anencephaly.info/pdf/anen-info_2006-USA.pdf )