We report a rare case of cystic intrapulmonary lymphangioma involving the left lung, which presented with pneumothorax and respiratory distress in a 6-month-old infant. Chest radiographs showed a multicystic lesion in the left lung mimicking the features of congenital cystic adenomatoid malformation of the lung. The lesion appeared on high-resolution CT (HRCT) as a multiseptate, air-filled cystic lesion in the left hilar area. Associated HRCT findings were thickening of interlobular septa and bronchovascular bundles in the left lung and the presence of peripheral pulmonary vessels within cystic lesions in the apex of the left lung. HRCT findings correlated well with histopathologic findings. We suggest that these associated findings may be helpful in distinguishing this condition from other cystic lung diseases and that this entity should be included in the differential diagnosis of multicystic lung lesions.
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