Pulmonary echinococcosis most frequently occurs in the right and both lower lobes. Children are more likely to develop pulmonary rather than hepatic echinococcal cysts. Some evidence suggests that echinococcal cysts develop more rapidly in the lungs of children than those of adults, which may explain the more common appearance of pulmonary cysts in children. Younger patients have larger cysts owing to their greater tissue elasticity. Large pulmonary hydatid cysts are critical because they pose a higher risk of rupture. Herein, we present an eight-year-old girl presented with severe respiratory distress due to giant hydatid cyst of right lung with shift of the mediastinum at left. The lesion displaced the trachea at left. The patient was successfully treated by parenchymal-saving operation. In conclusion, Giant hydatid lung cysts represent a distinct clinical entity most frequently encountered in children and adolescents with more serious symptoms. Although non-complicated giant hydatid cysts have a good prognosis regardless of their size and can be safely treated by parenchyma-preserving surgery.