Beckwith-Wiedemann syndrome consists of various abnormalities, including macroglossia, visceromegaly, omphalocele, and neonatal hypoglycemia. These abnormalities frequently require operative correction and careful anesthetic management. Principal problems associated with anesthetic management in this syndrome are congenital heart disease, hypoglycemia, and difficult airway combined with macroglossia. We report two cases of general anesthetic management in children with Beckwith-Wiedemann syndrome. (Korean J Anesthesiol 2009;56:583~6)