Alpha-1 Antitrypsin Deficiency (AATD) is an autosomal co-dominant condition. Airflow obstruction, emphysema, and bronchiectasis are reported as features of AATD. Pulmonary cysts have not been reported in this group of patients. We aimed to determine the presence/absence of pulmonary cysts in patients with AATD. We have recently set up a regional AATD centre in University Hospital Southampton NHS Foundation Trust, United Kingdom, and currently run this service in a combined clinical and research capacity. As part of cohort characterisation, we sought to determine associations with phenotype/genotype, smoking history, spirometry, and radiological phenotype in these patients. As an incidental finding, we identified pulmonary cysts on CT thorax of several patients with AATD, which were different to the cyst-mimics often seen in bullous lung disease associated with severe emphysema. The UHS AATD service currently has 84 patients, of whom 42 have ZZ phenotype/genotype, 18 SZ, and 24 MZ. 5 patients with AATD ZZ and 2 patients with AATD SZ had pulmonary cysts on thoracic imaging (13.5% and 22.2% respectively). There were no AATD MZ patients with pulmonary cysts. 3 patients with ZZ AATD and pulmonary cysts had never smoked, while 2 were ex-smokers. Of the 2 patients with AATD SZ disease and pulmonary cysts, one had never smoked and the other was an ex-smoker. There was no relationship between severity of emphysema and pulmonary cysts, although all but one of the patients with pulmonary cysts had mild bronchiectasis. Smoking was not associated with pulmonary cysts in our cohort. We propose that larger scale assessment of cysts in AATD be considered to establish AATD in the differential diagnoses in patients with cystic lung disease.