This chapter discusses the development of skull, pathology of various types of craniosynostosis and general principles of management. The craniosynostoses comprise a heterogeneous group of disorders of multifactorial origin. Most cases of simple craniosynostosis are sporadic. Apert’s and Crouzon’s syndromes are the common syndromic forms of craniosynostosis and characterized by brachycephaly and midface hypoplasia with syndactyly (Apert’s) or without syndactyly (Crouzan’s). Rapidly progressing hydrocephalus and ICP need to be attended prior to correction of synostosis. Early repair benefits from the pliability of infant skull. Later procedures are more for appearance.