Hepatolithiasis, or the presence of intrahepatic stones, is prevalent in East Asia and is characterized by the finding of stones within the intrahepatic bile ducts proximal to the confluence of the right and left hepatic ducts. Bile stasis and bacterial infection have been incriminated as the major aetiopathogenic factors. Clinical features include recurrent pyogenic cholangitis, multiple liver abscesses, secondary biliary cirrhosis and cholangiocarcinoma. The goals of management include accurate localization of pathologies, control of biliary sepsis and the elimination of stones and stasis. Ultrasonography, computed tomography and direct cholangiography complement each other in defining the stones, strictures and degree of liver damage. Non-operative biliary decompression by endoscopy and interventional radiology is effective in controlling the infection, but surgery remains the mainstay for the treatment of stones and strictures. Intra-operative ultrasound and flexible choledochoscopy, combined with percutaneous transhepatic cholangioscopy and intraductal lithotripsy, facilitate stone removal. Balloon dilatation and biliary stenting serve to open the bile duct strictures. The creation of a hepaticocutaneous jejunostomy after conventional surgery allows atraumatic access to the biliary system for the removal of recurrent stones.The management of biliary parasites begins with conservative measures, including analgesics and anti-helminthic therapy. In refractory cases or patients with acute cholangitis, endoscopic biliary drainage and the extraction of worms may be necessary. Improvement in sanitation plays a crucial role in the epidemiological control of these biliary diseases.