Cholangiocarcinomas still pose a therapeutic challenge for the surgeon, as there is no common entity for these diseases. Different histologies, tumor locations, and treatment techniques all affect the ultimate outcome. Multifocal (intrahepatic and extrahepatic) cholangiocarcinomas are rarely encountered and are often underestimated. Long-term survival is rarely achieved. We report on a 47-year-old woman with concomitant intra- and extrahepatic cholangiocarcinomas successfully treated by a combined Whipple's operation and left hepatectomy. Long term disease free survival was achieved without evidence of recurrence for 65 months to date.