A 77-year-old woman was admitted to Yeungnam University Hospital with jaundice and abdominal pain. Laboratory findings were as follows: total bilirubin, 9.0 mg/dL; aspartate aminotransferase, 199 IU/L; alanine aminotransferase, 120 U/L; alkaline phosphatase, 1,592 IU/L; γ-glutamyl transferase, 937 IU/L; and cancer antigen 19-9, 961 U/mL. An abdominal computed tomography (CT) scan revealed a 1-cmsized low-density mass in the perihilar area, with diffuse dilatation of the bilateral intrahepatic duct (Fig. 1C). Magnetic resonance cholangiopancreatography (MRCP) revealed a biliary stricture at the hepatic hilum, suggesting a Klatskin tumor (Fig. 1A). Endoscopic retrograde cholangiopancreatography (ERCP) was performed, and the findings were similar to those of MRCP (Fig. 1B). Following an endobiliary forceps biopsy, a nasobiliary drain catheter was inserted. The tumor stained positive for CD56, synaptophysin, and chromogranin (Fig. 2). Approximately 60% of cells were positive for Ki67 (Fig. 2D). These were compatible with a large-cell neuroendocrine carcinoma (LCNEC). The patient refused further treatment and was discharged following the placement of a 10 Fr 12-cm biliary plastic stent. One month later, she was re-admitted with abdominal pain. CT showed multiple new metastases in the liver and lymph nodes (Fig. 1D). She underwent 30 Gy/10 Fr palliative radiotherapy for 2 weeks. Despite radiotherapy, her condition deteriorated, and she was transferred to a nursing home for supportive care.