Focal dermal hypoplasia or Goltz syndrome is a rare clinical. entity,
usually presenting in early childhood as a mix of ectodermal and mesod
ermal anomalies. Previously reported cases have included skeletal lesi
ons of the long bones, metacarpals, metatarsals and pelvis. We present
a case with a vertebral lesion causing neurologic deficit. A discussi
on of the history of the syndrome is included as well as detailed hist
ologic description.