Abstract: Hydatid disease is a parasitic infestation caused by the larvae of the tape form Echinococcus. Cardiac involvement is rare, with a reported occurrence between 0.02- 2%. Primary involvement of the heart usually occurs via coronary arteries. Secondary involvement of the heart occurs from hydatid disease of adjacent organs, such as lungs or liver. Cardiac localization usually seen in adults and carries a significant risk of potentially lethal complications. Cardiac hydatid cysts are mostly intramyocardial. We present a 34-year-old woman with a hydatid cyst in the right ventricle. The diagnosis confirmed by echocardiographic, radiologic, serologic and histologic findings. Following albendazole therapy for 3 weeks, she was operated and a large alive cyst was removed.