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Cardiovascular hydatid disease is caused by parasitic infection of Echinococcus granulosus, which could be asymptomatic or life-threatening depending on lesion site, granuloma size, and disease progression. Diagnosis and treatment of cardiac echinococcosis should be under comprehensive consideration. In this case, we reported a successful right atrium-inferior vena cava bypass surgery in a 31-year-old female with unresectable right atrial echinococcosis and inferior vena cava obstruction.