Background: Bile duct carcinoma is an extremely aggressive and rare primary hepatobiliary malignancy affecting nearly 1-2/100,000 people in most countries in Europe. Biliary tract malignancies located in the distal third of the common bile duct (CBD) account for approximately 17-18% of all cholangiocarcinomas. This report aims to provide a step-by-step evaluation of a rare case of cholangiocarcinoma. Content: The current report focuses on a 65-year-old male patient who was presented to the emergency department with signs and symptoms suggestive for a biliary tract obstruction. An intense cooperation between multiple departments was required in order to perform a complete and accurate evaluation of the patient. Initially, the patient underwent an abdominal Ultrasonography (US), followed by an abdominal and pelvic Computed Tomography (CT), which revealed an intraductal tumor affecting the distal CBD. Furthermore, the patient underwent an endoscopic retrograde cholangiopancreatography (ERCP) in order to provide tissue samples and to perform a dilatation of the CBD in the affected area. The histopathology report confirmed the malignant nature of the lesion describing it as an adenocarcinoma. The surgeons performed a pancreaticoduodenectomy (Whipple procedure), managing to achieve negative tumor resection margins. However, the patient developed several complications that required multiple surgical reinterventions. Conclusions: This report presents a fully investigated less common type of cholangiocarcinoma, highlighting the principles of diagnosis and management as well as of a multidisciplinary approach in such patients.