This chapter presents a case study of a 3-month-old male who had an uncomplicated term delivery presented for repair of craniosynostosis. Premedication was avoided and a peripheral intravenous catheter was started because of the young age, presence of mid-facial hypoplasia, and concern regarding potential problems with ventilation and intubation. Probably the most challenging part of the anesthetic management of craniosynostosis repair is the significant blood loss and frequent rate of blood product transfusion. Craniosynostosis repair presents a number of challenges to the anesthesiologist: (1) small size of the patients; (2) significant and often unavoidable blood loss; (3) need for intraoperative transfusion of blood products; and (4) associated anomalies including airway problems and obstructive sleep apnea. All of these potential complications call for careful preoperative and intraoperative planning, meticulous attention to intravascular volume status and hemodynamic stability as well as maintenance of normothermia.