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Aims: We report herein an additional case of primary malignant fibrous histiocytoma (MFH) in the duodenum and provide a review of the existing literature., Methods and Results: A 61-yr-old Chinese man was admitted to our hospital with symptoms of melena, anorexia, and weight loss. An abdominal computed tomography (CT) and gastrointestinal barium meal examination demonstrated a tumor of the duodenum suggestive of primary malignancy. The tumor was successfully treated by pancreaticoduodenectomy. It was histopathologically and immunohistochemically diagnosed to be a storiform-type primary MFH of the duodenum. There have been a total of 40 cases of primary malignant fibrous histiocytoma of the small bowel documented in the literature including our Chinese cases., Conclusion: Primary malignant fibrous histiocytoma of the small bowel, especially in the duodenum is extremely rare. The final diagnosis is made only after pathological and immunopathological examination of the tumor. The malignant potential of such tumors is high. The prognosis may be mainly dependent on the invasion and metastasis of tumor, while tumor size is irrelevant. The treatment should be surgery if possible. Early surgical intervention may be the best form of management that may offer the patient good result.