Duplication of the extrahepatic bile duct (DEHBD) is a rare anomalous of the biliary system. Here we report two unique cases of DEHBD coexistent with hilar cholangiocarcinoma (Klatskin tumor). The two cases were diagnosed preoperatively and treated with a modified surgical technique. Case 1: A 77-year-old female presented with obstructive jaundice. Abdominal computed tomography (CT) scan revealed diffuse bile duct dilatation (BDD). Magnetic resonance cholangiopancreatography (MRCP) showed diffuse BDD, stone in the extrahepatic duct with distal common bile duct (CBD) stricture. Endoscopic retrograde cholangiopancreatography (ERCP) was attempted at other hospital and reported diffuse BDD. The procedure was aborted due to suspected contrast extravasation and the patient was transferred to our facility for further evaluation. Repeated ERCP revealed EHBDD with single biliary drainage to the duodenum (Fig. 1A). Findings were consistent with type Va EHBDD (Fig. 2). Brushing cytology was done. Rapid on-site evaluation (ROSE) by cytopathologist was suggestive of cholangiocarcinoma.Figure 1Figure 2Case 2: A 78-year-old female presented with obstructive jaundice for one month. Abdominal CT showed diffuse BDD. MRCP showed diffuse BDD to the level below CD take off. The patient had ERCP at other facility that showed distal CBD stricture. The stricture was dilated and stented with a 10 French x 5 cm PS. Brush cytology was negative. Patient's jaundice persisted; so she was referred to our institution for further evaluation. Endoscopic ultrasound showed hypoechoic and well-demarcated mass of 21.3x17.2 mm at the neck of pancreas. Fine needle aspiration was done and ROSE by cytopathologist was suggetive of cholangiocarcinoma. Old stent was removed. ERCP was repeated and showed type Va EHBDD(Fig. 1C). The biliary hilum was obstructed by the mass mimicking Klatskin tumor (Fig. 1C). In both cases, biliary strictures were dilated and stented with 2 plastic stents placed up to the right and left main extrahepatic bile ducts (Fig. 1B&D). Both patients were referred for pylorus-preserving Whipple surgery with hepaticojejunostomy. Intra-operative evaluation and surgical pathology confirmed the diagnosis of type Va EHBDD with cholangiocarcinoma involving a distally located biliary hilum (Klatskin tumor) (Fig. 3A&B).Figure 3Preoperative diagnosis of type V EHBDD coexistent with distal hilar cholangiocarcinoma (Klatskin tumor) is challenging and necessitates surgical approach modification. Instead of major hepatic resection or traditional Whipple procedure; Whipple procedure with hepaticojejunostomy was used to manage those potentially resectable Klatskin tumors in the setting of this anomaly.