A young patient presented himself to the emergency department with sudden-onset, breathing-dependent right-sided thoracic pain. The auscultation revealed diminished breath sounds on the right. The radiograph showed a pneumothorax which was immediately dealt with chest tube drainage. The CT scan of the thorax showed minuscule subpleural bullae. Video-assisted thoracoscopic surgery (VATS) was performed due to persistent fistulae formation through the drain. The subpleural, bullous and emphysematous changes were histologically confirmed. Investigations into the cause showed evidence of alpha-1-antitrypsin deficiency (AATD). The patient is a Pi MZ type. Few cases of spontaneous pneumothorax as the first manifestation of alpha-1-antitrypsin deficiency have been described. When diagnosing primary spontaneous pneumothorax, alpha-1-antitrypsin deficiency should be considered.

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