Despite the prevalence of hydatid cyst in humans, its occurrence in the heart is rare. The clinical signs of the mentioned cyst in the heart are diverse, which makes its diagnosis challenging. In addition, the cardiac hydatidosis is diagnosed late as this disease progresses gradually. In this report, the cases discussed of a case with intraventricular cardiac hydatid cyst with coronary artery disease and multiple hepatic hydatid cysts. After diagnosis, the patient underwent coronary artery bypass graft surgery, and the cyst was successfully removed. Considering the probability of heart involvement in endemic regions, devoting due attention to the disease as well as performing faster diagnosis of the disease can play a significant role in reducing its complications.