We report the case of a middle-aged woman presenting epigastric discomfort, hepatomegaly, biochemical signs of cholestasis, bone marrow plasmocytosis and Bence Jones proteinuria. Percutaneous liver biopsy disclosed kappa light chain deposition disease of the liver and fine needle aspiration of abdominal fat showed amyloid substance. Renal blood chemistries and urinalysis were repeatedly normal. To our knowledge, this is the first reported case of kappa light chain deposition disease of the liver and concomitant amyloidosis without renal involvement as the first manifestation of plasma cell dyscrasia. This condition should be considered in the differential diagnosis of intrahepatic cholestatic liver disease.