Key Clinical MessageCardiac hydatidosis is a relatively rare complication of echinococcosis. Understanding the atypical manifestations, potential associated risk factors, and epidemiology leads to optimal and timely management.AbstractCardiac hydatidosis is a relatively rare complication of echinococcosis, with a potentially life‐threatening condition. Here, we reported a large interventricular septal hydatid cyst bulging in the left ventricle accompanied by a huge cervical lamp with recurrent hepatic cysts that underwent cardiac surgery to excise the cyst uneventfully.