A patient with diffuse eosinophilic gastroenteritis, associated with progressive chronic interstitial pulmonary fibrosis, was followed for 29 years. His major symptoms were recurrent gastrointestinal bleeding, anemia, diarrhea, malabsorption, and increasing respiratory insufficiency. Multiple iejunal biopsies revealed eosinophilic infiltration and extensive villous atrophy. Results of a lung biopsy and pulmonary function tests were consistent with chronic interstitial pulmonary fibrosis. His gastrointestinal symptoms responded to steroid therapy, but he died of progressive respiratory insufficiency. At autopsy, villous atrophy was noted to extend throughout the whole extent of the small intestine in association with multiple “pseudopolypoidal” inflammatory lesions.