In our experience with five cases of Crohn's disease of the esophagus, the endoscopic appearance has been demonstrated. Corresponding to the basic pathological changes, the findings are very different, but two stages may be differentiated: Stage I in which inflammatory changes predominate as a mild or more often erosive-ulcerative esophagitis. Stage II is a stenosing form similar to a peptic stenosis or to a stenosing tumor. The morphological changes are predominantly limited to the lower part of the esophagus with a tendency to extend to the proximal regions. The diagnosis may be established endoscopically only in special cases with shallow ulcerations within a normal mucosa or with cobble-stone relief which is usually seen in the colon. In all other cases, a specific macroscopical appearance of Crohn's disease of the esophagus does not exist and no specific differentiation is possible from other forms of esophagitis. Only by a combination of endoscopy, radiology and histology can the diagnosis be suspected. Guided biopsies are not able to confirm the diagnosis histologically. The exact diagnosis of Crohn's disease of the esophagus is only possible by histological examination of the resected esophagus.