Cholangiocarcinoma is a malignancy of the biliary duct system that may originate in the liver and extrahepatic bile duct. Despite aggressive anticancer therapy and interventional supportive care, the overall survival is approximatively 6 months, most patients not being eligible for curative resection.Case description: A 64 year-old patient, diagnosed in 2008 with locally advanced bile duct neoplasm (T3N0M0), who underwent latero-lateral hepatoduodenal anastomosis and cholecystectomy in 2008, followed by neoadjuvant Gemcitabine chemotherapy and cephalic duodenopancreatectomy with tumor exeresis in 2009, uncompliant to follow-up, presents with abdominal meteorism, diffuse abdominal pain, inappetence, weight loss and increased fatigability. Initial ultrasonography showed a localized pancreatic tumor and liquid in the Douglas pouch, both confirmed by CT scan.Discussion: The 6-year survival compared to the general mean survival rate of 17 months, depending on the type of the neoplasm, raises questions about the otherwise histopathology confirmed diagnosis of well differentiated cholangiocarcinoma. The present image documented pancreatic tumor makes the retrospective diagnosis assessment even more intricate. Slow-progressor outlier cases might represent new diagnostic entities, and their understanding can be instrumental in improving the therapeutic efficiency. Long time survival in such diagnostic entities is scarcely reported in literature.