Ten children and adolescents (aged four to 21 years) with the homozygous form of alpha-1-antitrypsin deficiency (PiZZ) were investigated with regard to obstructive airway disease. Besides the recording of the history, tests of lung function and allergy tests were performed. It was shown that bronchial asthma occurred in three out of ten patients. However, we could not demonstrate any statistically significant increased incidence of bronchial asthma in our unselected PiZZ-patients compared to the overall population. There was no indication of emphysema in any of the nine patients.