Background <p>Cardiac echinococcosis is a rare but significant cause of mortality and morbidity, particularly in regions endemic to the Echinococcus parasite. Its high mortality rate, reported to be 15–20%, is mainly attributed to its associated severe complications. However, mostly the condition is asymptomatic until complicated or presented with sudden death.</p> <p>Main body.</p> <p>This article reports three different cases and studies fatal cardiac hydatid cysts in 38 articles which were eligible according to the inclusion criteria and involving 46 cases; their demographic distribution, the features of the cysts, other organ involvement, their presentation, and the cause of death. Males under 40&#xa0;years were the mostly affected by this disease, predominantly in regions such as Turkey, Tunisia, and Iran. Although the cysts were found in all cardiac parts, the left ventricle was the most commonly affected. Isolated cardiac cysts were found in over half of the cases, while the remainder had additional cysts in other organs, mainly in the liver, kidneys, and lung. At the end, hydatid cysts of the heart were found to cause death by arrhythmias, heart failure, anaphylactic shock, pulmonary hydatid embolism, or cerebral hydatid embolism.</p> Conclusion <p>This article highlights the importance of early detection of hydatid cysts of the heart, as many cases are diagnosed postmortem after sudden collapse and death. Early detection is crucial, and cardiac echinococcosis should be considered in the differential diagnosis of cysts that are found in any body part, especially in endemic areas. To our knowledge, this is the first review that focuses exclusively on autopsy-confirmed fatal cases of cardiac hydatid cysts, including a novel case series from Jordan. Furthermore, this review emphasizes the need for continued research to understand the mechanism of fatality, hoping that the current high mortality rate would be reduced significantly.</p>