Schwannomas of the rectum are uncommon and incompletely characterized tumours, and only a limited number of cases have been reported. On the basis of a case of rectal schwannoma and a review of the literature on this rare condition, we stress the importance of the clinical features, diagnostic difficulties and surgical indications for the various therapeutic approaches. The basis for radical operation, due to the tendency of such tumours to recur locally and the real possibility of malignant degeneration, is discussed. We also emphasize the difficulty of making a benign diagnosis with histological certainty.