Hydatid disease, also called cystic echinococcosis, is commonly by a tapeworm, Echinococcus granulosus. Frequently affecting the liver and lungs, involvement of other body organs has also been reported. Intracardiac hydatid cyst and involvement of interventricular septum is a relatively rare presentation. We report one such case of intracardiac hydatid cyst affecting the interventricular septum (IVS) at the apex of right ventricle (RV).