A case of diffuse primary amyloidosis with predominantly liver onset is reported. The absence of amyloid in the abdominal subcutaneous fat and in the rectal mucosa, the wide ECG voltage range, the absence of nephropathy and proteinuria as well as the absence of immunoglobulin involvement, are in contrast with autoptic findings of ubiquitous involvement of amyloid substance. It is considered that the pathogenesis of this disease is still open to discussion.