Cardiac involvement in hydatid disease is exceptional but has a formidable prognosis. Cardiac localization of hydatidosis exposes to the dissemination of vesicles in the systemic circulation, especially when it is located on the left side. Its treatment is essentially surgical, which helps to avoid complications and the spread of the disease. We report the case of a 13-year-old patient with no particular pathological history, hospitalized for an ischemic stroke and limb ischemia, who was found to have a mass at the apex of the left ventricle on transthoracic cardiac ultrasound. The diagnosis of hydatid cyst was strongly suggested by the discovery of hydatid cyst membranes during the anatomopathological examination of the operative samples from the surgical thrombectomy of the left lower limb. Cardiac magnetic resonance imaging showed a mycotic aneurysm, while serology for hydatid disease was negative. A medical treatment based on albendazole was initiated, but the patient died in a context of multi-visceral failure postoperatively after the cardiac mass excision performed under extracorporeal circulation.