Cholangiocarcinoma is a rare disease (0.15-0.16% in the general population). We present the case of a man, 64 years old, who was admitted to our clinic for emergency with intense jaundice, abdominal pain in the supra-umbilical region. Laboratory analysis revealed elevated total bilirubin (23.5 mg/dl), with predominant direct bilirubin and an increased serum level of alkaline phosphatase and GGT, AST, ALT. The abdominal CT shows an tumor infiltrating distal bile duct, with important dilatation of proximal biliary tree and enlarged retro pancreatic lymph node (8 mm). The first therapeutic procedure was an surgical exploration of the abdomen to asses the resectability of the tumor and an internal biliary drainage colecisto-gastrostomy to allow improving of patient's biological and clinical state and a latter radical operation, after remission of jaundice. After a month we performed cephalic duodenopancreatectomy. Pathology result: moderately differentiated adenocarcinoma of intestinal type (G2) pT2NOMO (stage II). The postoperative evolution was favorable encumbered by a small pancreatic fistula healed by conservative method.