Truncus arteriosus is a rare form of cyanotic congenital heart disease in which the embryologic separation of the arterial trunk remains incomplete. The primary anatomic lesion is a common arterial trunk that supplies the pulmonary, coronary, and systemic circulations. Patients become symptomatic early in infancy because pulmonary vascular resistance falls over the first days to weeks of life, resulting in pulmonary overcirculation. Ideally, truncus arteriosus repair occurs within the first weeks of life. The most common extracardiac congenital malformations associated with truncus arteriosus are those within the heterogeneous spectrum of 22q11 deletion syndrome. Features of 22q11DS can pose multiple challenges to anesthesiologists. Dysmorphic facies, including micrognathia, retrognathia, palatal abnormalities, and laryngotracheomalacia, can impede airway management. This chapter describes the care of a child with repaired truncus arteriosus and 22q11 deletion syndrome for palatal surgery and the perioperative management considerations.