Crohn's disease only rarely affects the esophagus, usually producing strictures and fistulas. Superficial lesions such as erosive esophagitis are infrequent. Histological proof of esophageal Crohn's disease is only exceptionally obtained with endoscopic biopsies. In a 4-year period we have followed 500 patients with Crohn's disease. Esophageal involvement was recognized in nine patients, usually because of painful dysphagia. Esophagoscopy revealed large aphthoid lesions. The clinical picture was characterized by: (a) involvement of multiple segments of the gastrointestinal tract, (b) extraintestinal manifestations, and (c) critical illness of the patients. Esophageal lesions and symptoms disappeared quickly with therapy. Routine histology of esophagoscopic biopsies revealed granulomas in only two patients, but additional sections showed granulomas in another five.