Holoprosencephaly is a rare disorder of embryologic development that denotes an incomplete or absent division of the embryonic forebrain (prosencephalon) into distinct lateral cerebral hemispheres. In most cases of holoprosencephaly, the fetus fails to survive to term, and those that do survive have severe functional limitations, including mental retardation. We present a case report of cranial vault remodeling in a 20-month-old female who was born with holoprosencephaly and a severe number 0/14 cleft. The patient developed chronic nonhealing ulcers secondary to hydrocephalus, ventriculoperitoneal shunts, and the underlying architecture of her cranial vault.