Biliary cystadenomas are rare, cystic neoplasms of the biliary ductal system that usually occur in middle-aged women. They cannot be safely differentiated from cystadenocarcinomas before operation and should always be considered for resection. Cystadenomas have a strong tendency to recur, particularly following incomplete excision, and a potential of malignant transformation. Therefore, complete resection is the therapy of choice and thorough histopathologic evaluation is imperative. A case of benign biliary cystadenoma with mesenchymal stroma is presented along with a review of the relative literature addressing the clinical presentation, histology, histogenesis, differential diagnosis, imaging features, treatment and prognosis of this interesting and rare entity.