Continuous advances in pediatric cardiology, surgery, and critical care have significantly improved survival rates for children and adults with congenital heart disease. Paradoxically, the resulting increase in longevity has expanded the prevalence of both repaired and unrepaired congenital heart disease and has escalated the need for diagnostic and interventional procedures. Because of this expansion in prevalence, anesthesiologists, pediatricians, and other health care professionals increasingly encounter patients with congenital heart disease or other pediatric cardiac diseases who are presenting for surgical treatment of unrelated, noncardiac disease. Patients with congenital heart disease are at high risk for mortality, complications, and reoperation after noncardiac procedures. Rigorous study of risk factors and outcomes has identified subsets of patients with minor, major, and severe congenital heart disease who may have higher-than-baseline risk when undergoing noncardiac procedures, and this has led to the development of risk prediction scores specific to this population. This scientific statement reviews contemporary data on risk from noncardiac procedures, focusing on pediatric patients with congenital heart disease and describing current knowledge on the subject. This scientific statement also addresses preoperative evaluation and testing, perioperative considerations, and postoperative care in this unique patient population and highlights relevant aspects of the pathophysiology of selected conditions that can influence perioperative care and patient management.