We describe herein the management of a 16-year-old girl with cystic echinococcosis of the right ventricle and massive obstruction of the pulmonary vessel system by parasitic metastatic dissemination. After resection of the cardiac cyst, pulmonary thromboendarterectomy was performed to remove parts of the obstructive parasitic material. The treatment reduced the elevated pulmonary arterial pressure, improving the patient's overall condition.