Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) can be a significant source of morbidity for pediatric cardiac patients in the intensive care unit. Children with unrepaired or palliated congenital heart disease are at increased risk for lower respiratory tract infections, while postoperatively they are at risk for ALI/ARDS precipitated by cardiopulmonary bypass. Surfactant dysfunction and inactivation are key contributors to the pathophysiology of ALI/ARDS, and there is growing evidence that exogenous intratracheal surfactant administration in noncardiac patients may ameliorate the lung injury seen in children with ALI/ARDS, leading to improved outcomes and survival. Evidence for a beneficial effect of exogenous surfactant treatment in congenital heart disease patients with ALI/ARDS is lacking. We present three consecutive children with congenital heart disease and ARDS who had significant and sustained improvement in lung function and oxygenation after treatment with exogenous surfactant, hastening their recovery from life-threatening hypoxemia. © 2010 the Authors. Congenital Heart Disease © 2010 Wiley Periodicals, Inc.