Liver transplantation in patients with end-stage hepatolithiasis is complicated by the high incidence of the suppurative cholangitis and systemic infection. A 43-year-old Korean-Japanese woman with hepatolithiasis, biliary cirrhosis, suppurative cholangitis, and biliary-bronchial fistula underwent living-related liver transplantation (LRLT) using a right lobe graft of her sister. The risk of infection was minimized by preoperative percutaneous transhepatic biliary drainage initiated 2 months before transplantation. The native liver was resected en bloc with the extrahepatic bile ducts and the infected section of the right hemidiaphragm. Opportunistic infection was prevented by limiting antimicrobial therapy to the interval from preoperative day 3 to postoperative day 4. Immunosuppressive agents were given below standard dose. The postoperative course following LRLT was uncomplicated, and hepatic function was good. Careful management of infection and adequate graft size are essential for successful LRLT in patients with end-stage hepatolithiasis.