A 24-year-old male was admitted for syncope and epigastric pain and fever. Biological examination showed leukocytosis and marked eosinophilia, the brain computed tomography (CT) was normal. The echocardiography revealed a voluminous mass in the free wall of the right ventricle compressing the right cavities without communication confirmed with thoracic CT. Hydatid serology was positive. Early and urgent surgery was decided. The postoperative period was uneventful and the patient was discharged home on albendazole. Hydatid cyst is an endemic parasitic infection in cattle-breading countries, and migration has played a significant role to expand this infection worldwide. Cardiac hydatidosis is rare (0.5-2%) in comparison with hydatidosis of the liver (65%) and lung (25%). The right ventricle location is even rarer, 10% versus 60% for the left ventricle, and can cause fatal complications such as anaphylactic shock, dissemination, and pulmonary embolism.