Abstract: Duodenal gastrointestinal tromal tumor(GIST) is a rare subset of stromal tumors arising from interstitial cells of Cajal. Despite developments in endoscopy, imaging technology, and immunohistochemistry, the diagnosis is difficult to confirm before the operation. Endoscopic ultrasound with fine needle aspiration is the gold diagnostic standard because of its high sensitivity and specificity. This technique is used to diagnose up to 80% of GIST cases. Surgical resection with microscopically clear resection margins is the only potentially curative treatment for nonmetastatic primary duodenal GISTs. Optimal therapeutic strategy of duodenal GISTs remains challenging because of the complexity of the pancreatico-duodenal regional anatomy. Recurrent risk and recurrence-free survival depend on tumor size, mitotic count, and NIH high risk classification rather than surgical approach. As a neoadjuvant therapy, Imatinib plays a key role in management of GISTs with recurrence and metastasis. The advances in the comprehension of the pathophysiology and treatment of GISTs may promote the advent of novel and effective treatment options.