Hepatobiliary cystadenoma is a rare tumor and has malignant potential. Owing to
its neoplastic nature, this tumor must be distinguished from simple hepatic cysts. Complete surgical resection is the recommended treatment. We report a 67-year-old woman presenting with right flank pain on urination for 4 months. Preoperative evaluation failed to provide definitive diagnosis. Enucleation of the tumor was done, and the pathologic report disclosed hepatobiliary cystadenoma of the liver. The patient was discharged 8 days after the operation and was followed up at our hospital for 22 months without evidence of recurrence. The literature concerning the entity is reviewed.