Abstract: Intrahepatic biliary cystadenoma (IBC) is a rare benign cystic neoplasm of liver, with malignant potential to transform into intrahepatic biliary cystadenocarcinoma. IBC predominantly occurs in women up to 85%, showing no special clinical symptoms and a polycystic lesion inside the liver on image examination. It is difficult to differentially diagnose from other cystic lesions of the liver such as simple liver cyst, intraductal papillary neoplasm of the bile duct, etc. The missed diagnosis and misdiagnosis rate were rather high in IBC, which is accurately diagnosed by pathological examination. Complete surgical removal of the tumor is the best way to cure it, and also can bring a satisfactory outcome to patients.