The congenital cystic adenomatoid malformation (CCAM) of the lung is a rare abnormality. The diagnosis may be discussed with many other broncho-pulmonary malformations and some acquired diseases.The aim of our work was to discuss some diagnostic and therapeutic problems of CCAM and prevent an unnecessary lobectomy.A retrospective study of 14 children operated over 20 years. We perform an analysis of personal cases of diagnostic and therapeutic errors.The preoperative diagnosis was established on clinical and radiological findings. The diagnosis of CCAM was dismissed by histology which found: interstitial emphysema (1 case), pulmonary infarction (3 cases), bronchiectasis (1 case), pulmonary capillary hemangiomatosis (1 case), lung abscess and unclassified lesions (7 cases) and hybrid lesion (1 case). In 1 case, the diagnosis of pulmonary abcess was rejected and CCAM was established.The diagnosis and the treatment of CCAM must be based on a multidisciplinary collaboration. The working diagnosis on clinic and radiology requires histological confirmation. Some diagnostic problems require confrontation with warned pathologists to avoid unnecessary lobectomy.