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We report a case of a 72-year-old woman who underwent a right hemicolectomy for a near-obstructing mass in the ileocecal valve. The histologic diagnosis was inflammatory fibroid polyp (IFP). IFPs are rare mesenchymal lesions that can arise throughout the gastrointestinal tract. Originally considered reactive or inflammatory, recent studies report activating mutations of platelet derived growth factor receptor-alpha (PDGFRA) in IFPs, suggesting that these may in fact be neoplastic. Nonetheless, these are benign lesions cured by local excision and typically do not recur or metastasize. Our patient also had no evidence of local recurrence one year after the surgery. Hence, the clinical importance lies in distinguishing these lesions from other benign and malignant mesenchymal proliferative lesions both on a biopsy and resection specimen. We review the literature on clinicopathologic features, immunohistochemical profile, pathogenesis, recent advances, differential diagnosis, prognosis, and treatment of these uncommon lesions., (© 2015 by the Association of Clinical Scientists, Inc.)