An unusual case of primary extranodal Hodgkin's disease, localized to the duodenum is reported and illustrated. A review of the literature stressing the incidence, clinical presentation, radiographic appearance and prognosis of primary gastrointestinal Hodgkin's disease is presented. It is pointed out that although the majority of the lesions are infiltrating in type, intestinal Hodgkin's may present as a solitary ulceration or as a polypoid lesion indistinguishable from other forms lymphoma.