The recent observation of a case of schwannoma in the second part of the duodenum lead the authors to a literature survey in order to state the present knowledge of this subject. Schwannoma is an ectodermal neoplasm arising from the nerve sheath that envelop axons. In the gastrointestinal tract these tumours can concern cells of the myentericus and submucosus plexus of the intestinal wall. A duodenal location is extremely rare. Gastroduodenal endoscopy, abdominal ultrasonography and CT scan were found as important investigatory tools, but only histological examination of the excised tissue suggests the definitive diagnosis. Therapy consists in the radical excision of the tumour; this is rarely obtained by endoscopic way because of the submucosal neoplastic growth. Then a surgical laparotomic approach is more often required and it allows to perform local excisions through a duodenotomy, partial duodenectomy or ampullectomy and more complex operations, like a cephalic duodenopancreatectomy, in front of extended papillar involvement. The latter was the suitable treatment for radical removing of the juxtapapillar schwannoma reported by the authors; the uneventful postoperative course, the patient's excellent general conditions and nutriture at 12 months confirm the validity of the therapeutic policy.