Pulmonary hypoplasia (PH) is a developmental anomaly of the lung parenchyma, characterized by a decrease in the number and size of airways, alveoli and vessels. We present a case of a 31-year-old patient with a history of chronic productive cough and frequent respiratory infections, who was referred for investigation of abnormal chest x-ray. The combination of chest computed tomography (CT) and bronchoscopy set the diagnosis of left pulmonary hypoplasia and the patient was treated surgically with a left pneumonectomy. PH is usually diagnosed immediately after birth, causing severe respiratory failure with high mortality. The less severe, unilateral forms can possibly survive by causing compensatory hyperinflation of the other lung and remain undiagnosed until adulthood, presenting either asymptomatic or with symptoms of chronic bronchitis and recurrent respiratory infections. Chest CT is considered the imaging technique of choice for the diagnosis and for the differential diagnosis from other congenital or acquired conditions. The treatment is usually conservative, although surgical resection is indicated in cases of severe cystic changes and intense symptomatology.

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