An intraductal papillary neoplasm involving the biliary tree is an unusual premalignant condition of epithelial origin, identified by its cystic dilatation of the biliary channels. Being a slow-growing tumor, surgery offers the best curative rate, especially in the setting of a low-grade disease. Here, we present a case of a localized, low-grade, intraductal papillary neoplasm of the bile duct (IPNB), residing in the liver, which was treated with resection of the liver lobe. The adjuvant treatment and prognosis highly depend upon the presence of dysplasia or a co-existent invasive malignancy. To the best of our knowledge, being a rare entity, this is the first case to be reported from Pakistan.