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Background: alpha-1-Antitrypsin deficiency (AATD) is a rare condition with a prevalence of about 1/4000 individuals in Germany. The clinical manifestations are the early development of pulmonary emphysema or of liver cirrhosis. The low prevalence of AATD suggested the establishment of a registry with the aim to learn more details about the natural history and the quality of care for these patients., Methods, Patients, Results: Since 2004 the German registry for individuals with AATD is maintained in Marburg. Up to May 2008, 548 individuals were registered, 455 with the genotype PiZZ and 46 with PiSZ. The age range of the registered individuals extends from 2 to 82 years (mean 55, SD 20; men 54 [SD 19] years, women 55 [SD 21] years). Most patients are affected by chronic obstructive pulmonary disease (COPD, distribution to the GOLD stages: GOLD I = 16 %, GOLD II = 30 %, GOLD III = 33 %, GOLD IV = 21 %). Currently 109 subjects are under substitution therapy. The time period between the first symptom and the establishment of the correct diagnosis was on average 5.6 years., Conclusions: The data of the registry allow a detailed characterisation of the natural course of the disease and the levels of the patient care.