Human hydatid disease occurs after infection with Echinococcus granulosus, mainly involved liver and lung. Here we reported a 48-year-old woman, who presented to our hospital with intermittent chest pain. Transthoracic echocardiography, Enhanced CT and MRI of heart were performed, revealed the cyst located at the interventricular septum near the right ventricular apex. Other organs including lung and liver were not involved. The cyst was successfully removed, while postoperatively pathological biopsy confirmed the infection of Echinococcus granulosus, thus, she was finally diagnosed as isolated cardiac hydatid cyst.