This article reviews the overall incidence of craniosynostotic anomali
es and provides an overview of the developmental aspects of these malf
ormations. Recent basic science investigations are examined, with part
icular emphasis on new genetic approaches that hold the key to unlocki
ng the causes and pathogenesis of craniofacial malformations. This inf
ormation ultimately will provide surgeons with a better understanding
of these anomalies and their classification, which has the potential t
o improve surgical results.