We report an unusual case of severe Crohn's disease with involvement of the lung. In 1993 at age 21 a male patient experienced a Helicobacter-negative duodenitis. A routine chest radiograph revealed spotted opacities in the right upper lobe without discomfort and normal pulmonary function except for a reduced diffusion capacity. Bronchoalveolar lavage showed mild lymphocytosis and biopsies from the macroscopically normal bronchi displayed non-caseating granulomas. In 1995 he had symptoms of peptic ulcer disease with a gastric outlet stenosis. He underwent Billroth I resection with only temporary improvement. Three months later the symptoms had recurred and a stenosing inflammatory process of the duodenum was seen on endoscopy suggesting Crohn's disease. A chest radiograph demonstrated infiltrations in parts of the left lung and bronchoalveolar lavage again revealed alveolar lymphocytosis (CD4/CD8 ratio 3.1). Both the gastrointestinal and pulmonary affections responded to corticosteroids but the duodenal stricture had to be resected. The association of Crohn's disease and pulmonary disorders is discussed.