Four cases presented unusual clinical manifestations due to biliary stone disease. Hepatomegaly and splenomegaly, weight loss, and fever, accompanied by hepatocellular damage were the presenting manifestations in all of the cases. There was a lack of typical symptomatology of gallstone disease in the history of the patients, though in every case, operation proved the presence of long-standing biliary tract abnormality. Malignancy or hepatitis were considered in the initial diagnosis in all of the cases. Liver function tests, scanning, and needle biopsy of the liver were of little diagnostic value in establishing extrahepatic biliary obstruction. The definitive value of transhepatic cholangiography in doubtful cases of hepatobiliary disease is emphasized. This procedure should be considered at a much earlier stage of the disease.