Alpha-1 antitrypsin deficiency (A1AD) is a rare genetic disorder characterized by early-onset emphysema and, rarely, liver disease and vasculitis. In the lungs, unopposed and enhanced elastase activity results in accelerated parenchymal destruction leading to emphysematous changes predominantly in the lower lung fields. Medical treatment includes standard therapies for emphysema and so-called “augmentation therapy” using purified pooled plasma alpha-1 antitrypsin. Surgical options include lung transplantation and lung volume reduction surgery. The option of lung volume reduction surgery potentially provides palliation of dyspnea and a bridge to lung transplantation.