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Background: Lemmel syndrome is a rare and misdiagnosed etiology of obstructive jaundice due to a periampullary duodenal diverticulum causing a mechanical obstruction of the common bile duct. It represents an obstructive jaundice with the absence of choledocholithiasis or pancreaticobiliary tumors. It is an underreported entity due to the absence of specific pathognomonic signs. Case presentation: A 77-year-old-woman admitted for sepsis, due to an ascending cholangitis, underwent a MRCP and a gastroduodenoscopy revealing Lemmel’s syndrome. Due to failure of ERCP, the patient underwent surgical derivation. Conclusion: Lemmel syndrome represents an uncommon diagnosis of obstructive jaundice, that shouldn’t be neglected if no other organic cause is detected. It is usually asymptomatic, however some patients can develop symptoms and complications such as cholangitis, as is the case of our patient. Imaging allows diagnosis, with MRCP as the modality of choice to confirm diagnosis. Endoscopy is the first line treatment.

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