摘要Malignant fibrous histiocytoma (MFH) occurs most commonly in the extremities and trunk, but rarely in the intestine. Here we report two cases of primary intestinal MFH. The first case was a 70-year old man admitted for recurrent right lower quadrant abdominal pain. At laparotomy, a tumor was found originating from the cecum, with a suspicious metastatic nodule on the surface of the right lobe of the liver. A right hemicolectomy was performed followed by an ileotransverse end-to-end anastomotic reconstruction.The second case was a 43-year old man with intussusceptions of the small intestine. An emergent laparotomy revealed 4 pedunculated masses in the small bowel and a partial resection of the small intestine was performed. Though the symptoms were not typical, based on histological and immunohistochemical studies, the patients were diagnosed as MFH of the intestine. They were not treated with chemotherapy or radiotherapy and both died within 3 mo. MFH of the intestine is an extremely rare neoplasm with an aggressive biological behavior. The pathogenesis of this disease has not been clarified to date. Complete surgical excision is preferred, adjuvant chemotherapy or radiotherapy may be advisable.