In a 37-year-old female patient with marked signs of cholestasis and excessive hyperbilirubinaemia without any recognisable cause, the unexpected diagnosis of massive amyloidosis of the peri-collagenous and perireticular type was made after hepatic needle biopsy. There were also amyloid deposits in the rectal mucosa and in the bone marrow. Fifteen months after the onset of disease symptoms the patient died from the consequences of spontaneous intrahepatic haemorrhage. At the post-mortem investigation generalised amyloidosis with massive involvement of nearly all organs was found. Primary amyloidosis should be considered in the differential diagnosis of any aetiologically unexplained cholestasis.