We report the case of a 62-year-old female patient operated for a hepatic hydatid cyst that years later was found to have a hydatid cyst in the inferior vena cava and right atrium that was the source of disseminated pulmonary spread of the disease and occlusion of the inferior vena cava blood flow. Cardiac hydatid disease is very uncommon and is frequently associated with a poor prognosis. The literature for hydatid disease of the heart is reviewed and the clinical and echocardiographic relevant findings of this patient are discussed.