The involvement of the upper gastrointestinal (GI) tract has been considered to be a rare manifestation of Crohn's disease (CD). Retrospective studies have reported prevalence figures of 0.5-13%. The diagnosis of CD of the upper GI tract is based on clinical, radiological, endoscopic and histologic features. In contrast to the retrospective studies, prospective studies, in which patients with CD underwent routine endoscopic evaluation with biopsies, revealed a much higher frequency of endoscopic and histologic abnormalities. Since Helicobacter pylori is the most frequent cause of gastritis and the most important etiologic factor in peptic ulcer disease, it is important to assess the contribution of H. pylori in the interpretation of the abnormalities observed in the upper GI tract in patients with CD. Therapy for CD of the upper GI tract consists of drug therapy and endoscopic or surgical interventions and is in fact similar to that for distal CD. Corticosteroids are still the most important drugs in the treatment of CD of the upper GI tract. Sometimes adjunctive therapy, e.g. gastric antisecretory drugs and mucosa protective agents, is beneficial. Endoscopic evaluation of the upper GI tract with biopsies should be part of the work-up of CD patients.