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Cardiac echinococcus is a rare affliction of the heart caused by the tapeworm Echinococcus granulosus. Primary echinococcosis of the heart represents 0.5–2% of all hydatid disease cases in endemic regions. It evolves slowly, explaining its rarity in children. We report the case of a 11-year-old child affected by a giant cardiac cyst of the left ventricle (LV). The patient underwent cardiac surgery and medical treatment. A retrospective review of the current literature was realized. We found 18 cases: the mean age was 11-years old. Nine cysts were localized in the LV, four in the interventricular septum, three in the right ventricle, and two in the right atrium. All underwent surgery except six patients. Routine echocardiographic screening may be useful in endemic regions where infestation is common. Cardiac echinococcus should be diagnosed in the early and uncomplicated stages and be removed surgically even in asymptomatic patients.