The major duodenal papilla is the terminal part of hepatopancreatic drainage. Variations in the bile duct and the main pancreatic duct opening are related to rotation and recanalization, which occurs during embryologic development. A double major papilla of Vater is a congenital anomaly that was rarely described before. We report a 49-year-old man with double papilla of Vater identified during Endoscopic intervention for obstructive jaundice. The patient had symptomatic gallstone disease and presented with biliary colic and obstructive jaundice. During duodenoscopy, the patient was found to have a double major papilla with a larger cranial one located at the expected usual anatomical position of ordinary cases. Canulation of this cranial papilla and injection of contrast medium identified an anatomically normal single common bile duct with stones and dilatation. Sphincterotomy and stone extraction was performed as usual. A literature review of this anomaly is presented. The clinical and endoscopic significance of this anomaly is discussed in detail.

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