Information on craniosynostosis in this paper updates “Craniosynostosis: Diagnosis, Evaluation, and Management” (Cohen MM Jr: New York: Raven Press, 1986). It also discusses recent developments that were included in the book but need further explanation or emphasis. Subjects discussed are: epidemiology, etiology, sutural biology, growth and development, neurological and psychosocial aspects, surgery, cloverleaf skulls, craniosynostosis syndromes, and prenatal diagnosis. Under the subject of etiology, fetal head constraint, maternal thyroid disease, calcified cephalohematoma, teratogens, and delayed suture closure and Wormian bones are considered. An updating of 15 cloverleaf skull conditions includes four monogenic disorders, two chromosomal disorders, one disruption, one iatrogenic condition, and seven syndromes of unknown cause. Newly recognized disorders with cloverleaf skull include Beare-Stevenson cutis gyratum syndrome and Say-Poznanski syndrome. Craniosynostosis syndromes and associations discussed include acrocraniofacial dysostosis, Apert syndrome, Beare-Stevenson cutis gyratum syndrome, Calabro syndrome, calvarial hyperostosis, chromosomal craniostenosis, Cole-Carpenter type osteogenesis imperfecta, Crouzon syndrome, Curry-Jones syndrome, Curry variant of Carpenter syndrome, cutis aplasia and cranial stenosis, Fontaine-Farriaux syndrome, Gomex-López-Hernández syndrome, Hersh syndrome, hyper-IgE syndrome and craniostenosis, hypomandibular faciocranial dysostosis, Marfanoid features and craniostenosis, Pfeiffer-type cardiocranial syndrome, Pfeiffer-type dolichocephalosyndactyly, and Say-Barber syndrome.