Echinococcus granulosus remains a clinical problem in undeveloped and developing countries. It commonly affects the liver and lung, but, rarely, other organs such as the heart can be involved. In this report, we describe an unusual case in which pericardial hydatid cyst mimicking acute coronary syndrome secondary to compression by pericardial hydatid cyst attached to the anterolateral wall of the left ventricle. We made diagnosis by transthoracic 2D echocardiography, and confirmed by cardiac magnetic resonance imaging and serologic tests. Because our patient refused to have an operation, albendazole therapy was given. It is important to recognize this unusual disease, because it may lead to serious complications.