Craniosynostosis is the premature fusion of one or more of the cranial sutures. About 8% of the patients have familial or syndromic forms of synostosis, and in the remainder it occurs as a spontaneous isolated defect. Familial craniosynostosis syndromes are typically transmitted as an autosomal dominant trait resulting in disruption of the fibroblast growth factor receptor pathway. Familiarity with the characteristic head shapes resulting from craniosynostosis allows bedside diagnosis and differentiation from positional plagiocephaly. Because of the risks associated with untreated craniosynostosis, surgical treatment is usually undertaken soon after diagnosis. Current surgical methods include open calvarial reconstruction, minimally invasive strip craniectomy with use of postoperative molding helmet, minimally invasive strip craniectomy with spring implantation, and cranial distraction. Early referral to a pediatric craniofacial center allows all treatment options to be explored.