Inflammatory fibroid polyp, a rare, benign lesion of the gastrointestinal tract, arises from the submucosa. It consists of loose fibrous stroma of stellate cells and thin-walled blood vessels infiltrated by inflammatory cells, especially eosinophils. 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 extramural component, which has not been described previously. Clinically, symptoms vary and are nonspecific. Inflammatory fibroid polyp must be considered in the diagnosis of polypoid and/or extramural masses of the gastrointestinal tract, especially of the stomach and ileum.