Abstract: Objective To report a case with giant polycystic liver disease treated by liver transplantation. Methods A 38-year-old woman admitted to our department on September 4, 2017 for " 5-year history of polycystic liver disease and refractory ascites for 5 months". The patient underwent liver transplantation on September 14, 2017. Results The operation was successful. The size of resected liver lesion was 57 cm×18 cm×9 cm, and the weight was 10.6 kg, which made it difficult to expose the hepatic hilar region. Totally, 18 000 ml ascites was drained. The patient recovered well and was discharged on the 22nd day after operation. She was followed up for 19 months with stable liver function. Conclusion Liver transplantation is the best treatment for patients with polycystic liver disease, with good prognosis.