Inflammatory fibroid polyps are rare, benign, tumor-like lesions of the gastrointestinal tract. The lesion was first described by Vanek in 1949. Most frequently, they are localized in the gastric antrum, but can develop anywhere in the gastrointestinal tract. In the small intestine, these polyps cause obstruction-intussusception. Most IFPs are polypoid masses smaller than 5 cm, although sizes up to 20 cm have been reported. We report a case of a 56-year-old man with progressive midabdominal pain and tenderness in left lower quadrant. CT scan of abdomen and pelvis revealed an unusual, tubular, elongated mass causing mild partial obstruction and short segment intussusception. The patient underwent exploratory laparotomy and resection of the mass. Gross examination showed a large, solid, tan-white elongated tumor mass protruding into the small bowel lumen on one side and pushing the serosal surface on the other side. The large luminal portion of the protruding tumor measured 15.0 × 5.5 × 4.0 cm, and the extraluminal portion measured 5.5 × 4.0 cm. Total length of the tumor was 20.5 cm. Multiple sections showed a well-demarcated, nonencapsulated, fibrohistiocytic mass composed of widely spaced, bland, spindled and stellate-shaped cells emdedded in a collagenous fibromyxoid stroma with trapped collagen bundles, eosinophils, and few scattered plasma cells with foci of benign lymphoid aggregates. Immunoperoxidase stains were positive for CD34, vimentin, and CD68 (fibrohistiocytic marker) and negative for c-kit (CD117), actin, desmin, and S-100 protein. There was no histologic evidence of malignancy. The histopathologic features and the immunoperoxidase stains were consistent with a benign, nonneoplastic tumor-like mass, consistent with a giant inflammatory fibroid polyp-tumor-like mass (Vanek tumor). Inflammatory fibroid polyp, a rare, benign lesion of the gastrointestinal tract, arises from the submucosa. The lesion described in the present report is the largest inflammatory fibroid polyp reported to date; it occurred in the ileum, heretofore considered a rare location, and had a marked intraluminal and extraluminal component.