Introduction: We aimed to present a case of acute coronary syndrome with unexpected etiology complicated by syncope and arrhythmias, confirmed by imagistic examinations as cardiac parasitosis. Cardiac parasitic diseases are rare diseases, whose diagnosis and therapy should be adapted to each case. Imaging techniques allow precise diagnosis of cardiac echinococcosis, providing essential structural details on the damage degree of heart structures, allowing optimization of complex treatment in these cases. Case presentation: A 67-year old, obese and diabetic woman presented with cardiac syncope, arrhythmias and acute chest pain. Imagistic examinations excluded intracoronary thrombosis and confirmed a severe structural damage of myocardial tissue, consisting in replacement of the myocardial structure by many cysts caused by parasitic infestation with echinococcus multilocularis and echinoccocus granulosus originating from the liver. CT scan confirmed severe distruction of the left ventricular myocardium by policysts, that led to thinning of inferior and apical left ventricle wall without any possibility of surgical excision. Therefore a specific chemotherapy with albendazole was initiated. Follow up at 2 months indicated a favorable evolution, with serological decrease of echinococcal antibodies and reduction of cysts volume. Conclusion: In cases of angina and arrhythmias with non-atherosclerotic etiology, imaging techniques can diagnose the anatomopathological substrate of the disease and represent a valuable tool for the follow up.