Hydatid cyst can simultaneously affect the liver and lung. Some patients might have additional comorbidities that can make management more challenging. Here, we present a 10-year-old boy with hepatopulmonary hydatid cysts and severe mitral regurgitation, who was successfully managed with a staged surgical approach treating the lung first, followed by the liver and finally, the heart. © The Author [2008]. Published by Oxford University Press. All rights reserved.