Systemic amyloidosis presenting with jaundice is rare. A case of prima
ry amyloidosis presenting with severe intrahepatic cholestasis is repo
rted. The patient had hepatomegaly, ascites, and a markedly elevated s
erum alkaline phosphatase level. He had a rapid downhill course result
ing in death. Antopsy showed evidence of amyloidosis involving multipl
e organs, including the liver, kidney, and heart.