Hepatolithiasis, or primary intrahepatic stones, is prevalent in the Far East. This clinical syndrome has been previously referred to in the West as Oriental cholangiohepatitis. The majority of primary intrahepatic stones are calcium bilirubinate stones, but intrahepatic stones with high cholesterol purity have recently been recognized. Primary intrahepatic stones are formed de novo within the liver and can be distinguished from extrahepatic stones on the basis of a unique pathogenetic etiology, chemical composition, and clinical course. Patients with hepatolithiasis are often plagued by a progressive illness punctuated by multiple intrahepatic strictures, recurrent cholangitis, liver abscesses, 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.