Intraductal papillary neoplasm of the bile duct (IPNB) is a rare premalignant condition, defined as an epithelial neoplasm of the bile duct with exophytic papillary growth that can develop in any segment of the biliary tree. This pathology, with the highest prevalence in Asia, has been associated with the presence of hepatolithiasis and eastern liver infection (clonorchiasis). The diagnosis will depend on the clinical suspicion against the findings evidenced in the different diagnostic supports (invasive and non-invasive). Curative resection with negative margins is the treatment of choice in patients candidates for surgical management. In the event that the patient is not a candidate for curative resection, palliative treatment includes chemotherapy, percutaneous and endoscopic drainage, laser cholangioscopy ablation and intraluminal therapy with iridium 192. We present two cases of two patients with a diagnosis of IPNB confirmed by histology treated at our institution. The first case in an 86-year- old patient had a history of recurrent obstructive biliary syndrome and clinical suspicion of a new episode of cholangitis, and the second case in a 73-year-old patient who had a disseminated infectious process (spondylodiscitis, pelvic and intra-abdominal abscesses), and with the clinical suspicion of presenting a primary hepatobiliary focus. Both patients underwent single-operator cholangioscopy plus biopsy, confirming the diagnosis by histology. The first case was managed with palliative intent, indicating endoscopic diversion of the bile duct, while surgical management was indicated in the second case.