We report a 50-year-old woman with alpha 1 antitrypsin deficiency (PiSZ phenotype) who developed clinical features of malabsorption after vagotomy and pyloroplasty. The intestinal biopsy demonstrated total villous atrophy with hypertrophy of the crypts. After gluten withdrawal, digestive function and the appearance of the intestinal biopsy returned to normal. We raise the possibility of an association between alpha 1 antitrypsin deficiency and celiac sprue.