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Hydatid disease, also known as cystic echinococcosis, is a parasitic infection caused by the larval stage of Echinococcus granulosus. It usually invades organs such as the liver and the lungs but can affect any organ. Isolated cardiac involvement is a rare manifestation. We present a case of isolated left ventricle hydatid cyst with negative serological tests, which was treated with surgical removal and confirmed by histopathological examination.