For patients with COPD, FEV1 has been shown to be the single most important factor to influence survival. Patients with alpha 1-antitrypsin deficiency (alpha 1ATD) develop emphysema at a young age, especially if they smoke, and the purpose of this study was to calculate survival with respect to the initial FEV1 and smoking history. For this study, patients with Pi-type ZZ were analyzed. FEV1 was divided in four groups: below 25%, 25 to 49%, 50 to 74%, and more than 75% of predicted. A total of 347 patients with 107 deaths were available for analysis. Three patients were lost to follow-up, and 17 had received a lung transplant. There was no significant difference in survival between males and females. The median survival time for patients with an initial FEV1 below 25% was 6.3 yr (95% CI 5.2-8.0). For the FEV1 groups 25 to 49% and 50 to 74%, median survival times were: 10.5 (9.2-11.9) and 14.2 (12.4-infinity) yr, respectively. The median survival for the above 75% group was not calculable. For patients with an initial FEV1 less than 50%, smokers had a poorer survival rate than patients who quit smoking during the study period (p < 0.01). We conclude that for patients with severe alpha 1ATD, FEV1 is a very strong predictor of survival regardless of sex. Patients who quit smoking had a better survival rate than patients who continued to smoke.