1 Introduction
Primary cystic hepatobiliary neoplasm is a rare entity that can be
benign and malignant.1 The overall risk of malignant
transformation or even harboring malignancy warrants careful evaluation
and timely management of these cysts. Biliary cystadenoma/ mucinous
cystic neoplasm and intraductal papillary mucinous neoplasm of bile duct
(IPMN-B) are two broad categories of benign/premalignant
cysts.1 Cystic IPMN-B is a recently described distinct
entity occurring exclusively in intrahepatic bile ducts. The risk of
malignant transformation is 64% to 89% compared to 2% to 30 % in
biliary cystadenoma.1 However, making the clinical
diagnosis of these cysts is a diagnostic challenge. We present a unique
case of cystic IPMN-B of the left lobe of the liver with associated
liver atrophy in an elderly lady.