Congenital pulmonary airway malformations (CPAMs) are a heterogeneous group of hamartomatous cystic and noncystic lung lesions that result from early airway maldevelopment. Usually they are distinguished according to Stocker's classification in type 0, 1, 2, 3 and 4. We present the case of a 2 weeks old baby who was admitted to hospital with RDS symptoms and left pleural effusion: X rays and CT were suggestive for a pulmonary cystic lesion with pleural complications. Because of the persistence of pleural empyema and the development of a pneumothorax the baby underwent surgery. The histological examination revealed a type 3 CPAM associated with pleural loculated empyema. According to this case, in newborns with RDS loculated pleural empyema may mimick pulmonary cystic lesions; a treatment-resistant pleural empyema or pyopneumothorax in a newborn can recognize a CPAM 3 as a probable underlying condition, even in the absence of lung suppurative changes; CPAM 3 involving only two lung segments can have an excellent prognosis after surgical excision.