BACKGROUND:?Carolis disease, a rare congenital hepatic disease, has a poor prognosis, especially in patients with diffuse dilatation of the bile ducts. But liver transplantation has been a curative option. The aim of this study was to investigate the feasibility and rationality of orthotopic liver transplantation as an indication for patients with diffuse Carolis disease. METHODS:?The data from 3 patients with diffuse Carolis disease who had undergone orthotopic liver transplantation in our unit were analyzed retrospectively. RESULTS:?On postoperative day 7, patient 1 had acute rejection which was relieved after pulse treatment with methyprednisolone. He was discharged from hospital on postoperative day 27 and has been in good health for 82 months. Patient 2 had no acute rejection or severe complications, but died of chronic graft dysfunction 34 months postoperatively. Patient 3 had acute rejection on postoperative days 10 and 35, complicated with pulmonary infection, pleural effusion and opportunistic infection. After successful treatment, she resumed work and has been followed up for 47 months. Her condition is good. CONCLUSION:?Liver transplantation can offer an effective therapy for patients with diffuse Carolis disease, and can provide satisfactory long-term results.