Young-Simpson Syndrome (YSS) is a rare malformation syndrome characterized by facial dysmorphism, congenital heart abnormalities, congenital hypothyroidism and severe growth retardation. A 5-month-old girl was scheduled to undergo patch closure of atrial septal defect. She had been diagnosed with YSS preoperatively. We report out clinical experience of a case of YSS patient with brief review of related literatures and relevant anesthetic problems.