We report a case of sarcoidosis with severe cholestasis and cholangiographic features of sclerosing cholangitis that responded dramatically to corticosteroid therapy. Although an association between sarcoidosis and primary sclerosing cholangitis has been suggested by previous reports, features suggestive of primary sclerosing cholangitis, including inflammatory bowel disease, hepatic histology and serum neutrophil cytoplasmic antibodies, were absent in this case. Cholangiography may be useful in the evaluation of patients with cholestatic sarcoid liver disease, and intrahepatic biliary strictures should be included in the spectrum of hepatic involvement by sarcoidosis. A trial of corticosteroid therapy may be of benefit in patients with bile ductal involvement by sarcoidosis.