Improvements in the diagnosis and treatment of congenital disorders have resulted in a change in surgical practice. Many conditions that formerly required corrective surgery immediately after birth are no longer surgical emergencies. Most babies with congenital anomalies that can be corrected by surgery are now stabilized and optimized before the procedure. This article focused on the more common conditions that require semi-elective or urgent surgery in the neonatal period. Salient features of each of these disorders were described. Factors unique to each of these conditions that can affect the anesthetic course of these children were discussed. Methods and techniques that may aid in the anesthetic management of these children were delineated.