Lesions of the ileocecal valve are uncommon and include various inflammatory diseases, submucosal fatty infiltration, edema, and neoplasia. Of the neoplastic lesions, adenomatous polyps are distinctly rare, despite their frequency in the remainder of the colon. Three patients with adenomatous polyps of the ileocecal valve are added to the seven previously reported cases. One had a large pedunculated polyp, another had polyps resulting from familial polyposis, and the third had circumferential adenomatous polypoid hyperplasia and the ileocecal-valve syndrome. Although many lesions of the valve produce the characteristic symptoms, the diagnosis of the ileocecal-valve syndrome is seldom made. Most ileocecal-valve lesions are incidental findings on barium-enema studies and may be carefully followed. The indications for operative intervention are suspicion of malignancy and the presence of troublesome symptoms. If malignancy is likely, a right colectomy should be done. Segmental resection is the procedure of choice for the ileocecal-valve syndrome. Adenomatous polyps may require simple polypectomy, segmental resection, or even subtotal colectomy. Following recognition of these unusual disorders the exact management is individualized.