Despite the fact that hepatic involvement is frequently seen in systemic amyloidosis, major clinical symptoms or impaired hepatic functional capacity are rare. We describe a patient with primary hepatic amyloidosis, severe intrahepatic cholestasis and portal hypertension, a combination previously reported only three times in the literature. In case of an unexplained intrahepatic cholestasis or portal hypertension the possibility of amyloidosis should be considered and a Congo red staining should be performed.