Abstract
Biliary cystadenoma and cystadenocarcinoma are rare cystic liver tumours arising from the bile ducts proximal to the hilum of the liver. The tumours have unique structure with ovarian-type mesenchymal stroma, leading to challenging considerations about their pathogenesis. The differential diagnosis is wide and further complicated by the lack of awareness about these neoplastic processes due to the rarity of the disease. Radical surgical treatment can be recommended whenever possible as it can result in prolonged survival.