[Upper abdominal pain and febrile episodes in a 44-year-old Filipino woman].

A 44-year-old Filipino woman presented with abdominal pain and fever. Clinical examination and blood tests revealed no pathological results; however, (cross-sectional) imaging showed saccular cystic bile duct dilatation in the right liver with solid intraductal masses. Due to the clinical presentation the patient was admitted for surgical intervention with the diagnosis of Caroli disease. During the surgical procedure histopathology showed an intraductal papillary neoplasm of the bile duct (IPNB). The planned segmentetomy was extended to hemihepatectomy. IPNB is a rare entity of premalignant lesions of the bile duct system first recognized by the World Health Organization in 2010.