We report a case of bilateral and multilobar congenital cystic adenomatoid malformation (CCAM) in a four-months-old child with good clinical results after resections of the lesions. This is a relatively rare form of pulmonary disease. The final prognosis in these patients depends on the type of malformation, the presence or absence of fetal hydrops and on the extent of affected lung. Few cases of multiple involvement have been reported. We will consider the physiopathological aspects of the case, late clinical presentation and treatment and the positive surgical response based on the findings of the functional and anatomic imaging studies.
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