Vanishing lung syndrome (VLS) is also referred to as idiopathic giant bullous emphysema and is a rare manifestation of chronic obstructive pulmonary disease (COPD). Middle-aged tobacco smokers, younger marijuana users, and those with alpha-1-antitrypsin deficiency may especially be affected. The clinical and radiographic findings of VLS may initially be misinterpreted as spontaneous pneumothorax. High-resolution CT is the diagnostic imaging modality of choice in these patients and can help to differentiate VLS from pneumothorax. Such imaging also helps guide appropriate management. Here, SERPINA1 is linked to chronic obstructive pulmonary disease.