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The authors report an exceptional case of acute abdomen caused by the necrosis and perforation of a duodenal duplication cyst in a 23 year-old-patient. The cyst was located at the anterior and lateral surface of the duodenum, between its first and second portion. The patient underwent a cephalic duodenopancreatectomy (Whipple's procedure) because of the intimate adherence of the necrotic and inflammatory mass to duodenum and head of the pancreas. The histological examination of surgical specimen confirmed the diagnosis, showing a cystic structure lined with an epithelium and a common muscle wall shared by the cyst and the duodenum. There was no communication between the cyst and duodenal lumen.

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