A 66-year-old woman was admitted to the hospital with a neurological syndrome characterized by ataxia and diplopia. Magnetic resonance of the brain documented an ischemic medullary lesion. Examination of the heart revealed a grade 2/6 systolic murmur at the left sternal border and apex that radiated towards the axilla. The echocardiography study showed a mild mitral regurgitation, and an abnormally enlarged posterior papillary muscle was found in the left ventricle. Two weeks after admission, the patient died suddenly due to a stroke. At autopsy, a recent vast swollen ischemic cerebral infraction was found. Furthermore, two old infarcts were observed in the spleen. A cardiac examination showed a white-gray ulcered mass in the left ventricle and histological analysis revealed a rhabdomyosarcoma.