The authors report a case of hereditary amyloidosis in a 54-year old patient with an essentially neurological clinical expression. The cardiovascular assessment, consisting of echocardiography performed systematically while the patient was free of any cardiac symptoms, revealed typical amyloid infiltration with a hyperechoic, shiny appearance of the myocardium and significant parietal hypertrophy. Systolic function was preserved, in contrast with impairment of diastolic function, revealed by the presence of Appleton type I mitral blood and decreased propagation velocity of the transmitral flow on colour TM. The authors stress the importance of ultrasonographic examination in all patients with suspected cardiac amyloidosis, even in the absence of clinical or electrical signs.