Echinococcosis is a frequent parasitic human infection in sheep-farming areas. It is caused by the larval or the cyst stage of a tapeworm, mainly Echinococcus granulosis. Humans can be infected by ingesting tapeworm eggs, from which cysts will be developed mostly in the liver and the lung. Cardiac involvement of echinococcosis is rare and its clinical evolution is silent till the complication stage. A young adult died suddenly. The autopsy showed a ruptured hydatid cyst hollowed on the right side of the interventricular septum, protruding in the ventricle. The left pulmonary artery contained white-colored fragments of a membrane, similar to the one found in the right ventricle, associated to small vesicles. All these elements were obstructing this vessel, extending to small pulmonary arterial branches. Dissection of the other organs did not show other locations. Microscopic examinations ascertained the diagnosis of echinococcosis. Death was imputed to a right ventricular hydatid cyst rupture with pulmonary artery embolism.