Pulmonary cysts in BHD are elliptical and lentiform-shaped, often subpleural, and distributed predominantly in the lower lung zones [6,10]. Emphysema due to alpha-1 antitrypsin deficiency and infectious causes like Pneumocystis carinii can also mimic the appearance of cystic lung diseases, and the medical team should be mindful of them too. The gene discussed is SERPINA1; the disease is pulmonary emphysema.