Sarcomas rarely arise in the intestinum, and leiomyosarcoma represents the majority of cases. With only seven cases reported in the available English literature up to now, malignant fibrous histiocytoma of the small intestine is exceedingly rare. Moreover, follow-up data are almost completely unavailable. We present herein the unique case of a malignant fibrous histiocytoma arising in a postoperatively adherent intestinal loop. To the best of our knowledge, this is the first such case ever to be described. The clinical history and the intraoperative findings suggested that chronic postoperative repair processes might have been a promoting factor in the tumorigenesis of this neoplasm, on the analogy of malignant fibrous histiocytoma arising at different sites. The patient recovered well but 9.5 years after surgical removal, a solitary recurrent tumor developed in the urinary bladder and progressed rapidly, highlighting the need for long-term, possibly life-long, surveillance of patients with this rare type of intestinal cancer.