Hepatolithiasis, or primary intrahepatic stones, is prevalent in the F
ar East. This clinical syndrome has been previously referred to in the
West as Oriental cholangiohepatitis. The majority of primary intrahep
atic stones are calcium bilirubinate stones, but intrahepatic stones w
ith high cholesterol purity have recently been recognized. Primary int
rahepatic stones are formed de novo within the liver and can be distin
guished from extrahepatic stones on the basis of a unique pathogenetic
etiology, chemical composition, and clinical course, Patients with he
patolithiasis are often plagued by a progressive illness punctuated by
multiple intrahepatic strictures, recurrent cholangitis, liver absces
ses, and hepatic destruction. Advances in interventional radiological
techniques, cholangioscopy, and novel surgical innovations have led to
significant changes in the way these patients are now managed.