A 67-year-old Asian man presented to our hospital with yellow skin and dark urine. At first, he found his eyes were yellow 1 month ago. Being yellow extended to his face and trunk which was accompanied by dark urine. He denied any fever, chills or abdominal pain. During the past 6 months, he had lost 7kg of weight. His past medical history was remarkable for total knee replacement 5 years ago. Vital signs were within normal limits. A physical examination revealed a chronically ill appearance and jaundice. There was no evidence of abdominal distension, palpable mass, or organomegaly. His laboratory test results were as follows: aspartate aminotransferase, 260U/L (normal range 5 to 40); serum glutamic-pyruvic transaminase, 567U/L (normal range 5 to 40); gamma-glutamyl transferase, 1504U/L (normal range 16 to 73); total bilirubin, 20.3mg/dL (normal range 0.2 to 1.1); direct bilirubin, 13.7mg/dL (normal range 0 to 0.6); alkaline phosphatase, 924U/L (normal range 42 to 128); and cancer antigen 19–9 (CA19-9), 125.1U/mL (normal range 0 to 33). Abdominal ultrasonography revealed a tubular mass in the distal common bile duct (CBD), dilated intrahepatic bile ducts, and a distended gallbladder. An abdominal computed tomography (CT) scan showed a low-density mass at the common hepatic duct and the distal CBD with dilatation of the intrahepatic duct and his gall bladder. Positron emission tomography (PET) revealed two tumors: one proximal tumor with a maximum standardized uptake value (SUV) of 8.5 and a distal tumor with a maximum SUV of 7.1. We concluded that the tumors were synchronous double primary cholangiocarcinomas and decided to perform pancreaticoduodenectomy. There was no evidence of anomalous pancreaticobiliary duct union in CT or endoscopic retrograde cholangiopancreatography. In the operative fields, there was no evidence of distant metastasis. Two cholangiocarcinomas were removed with negative surgical margin. The cut surface revealed a gray-white colored, irregularly elevated, firm mass with ulceration in the distal CBD, measuring 17×15mm. On microscopic examination we found a moderately differentiated adenocarcinoma which invaded the fibromuscular layer of the CBD (stage T1b), metastasized to one regional lymph node (N1), and showed multiple lymphovascular tumor emboli. In addition, another mass was present just below the bifurcation of his hepatic duct. It was gray-white in color and measured 15×10mm. Sections from this specimen revealed a well-differentiated squamous cell carcinoma confined to the fibromuscular layer (stage T1b, Figure ). In addition to the distinct localization of these tumors, there was no transitional area between the two lesions and no intermingled histological features favoring a diagnosis of adenosquamous cell carcinoma. Taking all of these findings into consideration, the diagnosis was synchronous double adenocarcinoma and squamous cell carcinoma of the bile duct. The patient displayed a good clinical course and was discharged 23 days after surgery. However, 3 months later, multiple liver metastases were detected. He refused treatment and died 8 months after the operation.