Gastrointestinal stromal tumors (GISTs) are uncommon tumors that arise mostly in the gastrointestinal tract. Duodenal GIST (DGIST) comprises 4.5% of all GISTs. Surgery is the mainstay of treatment for localized, resectable GISTs although imatinib mesylate, a tyrosine kinase inhibitor, is effective for GISTs. In this study, we describe a case in which segmental resection of the third portion of the duodenum, have been performed for the treatment of a DGIST. Recent anatomical knowledge of the head of the pancreas has facilitated duodeno-pancreas surgery and duodenal resection preserving the pancreatic head can now be performed safely for larger tumors which are located below the papilla of Vater in the third or fourth duodenal portions with end-to-end or side-to end duodeno-jejunal anastomosis. Local resection can be beneficial for patients because it contributes to a better quality of life and does not involve the excessive resection associated with duodenopancreatectomy.