Mucinous cystic neoplasia of the liver and biliary tract represents a rare entity characterized by peculiar morphology, most commonly found in adults, particularly women between 40 and 50 years of age. Clinically, these tumors may cause abdominal pain, compressive symptoms, and jaundice. In 85% of cases, the lesion develops in the liver, while involvement of the extrahepatic bile ducts and gallbladder is even rarer. The differential diagnosis between cystadenoma and cystadenocarcinoma is challenging in the preoperative setting; therefore, surgical resection with margins is recommended due to the risk of malignancy.