Pleuropulmonary blastoma PPB is an extremely rare, intrathoracic neoplasm of early childhood with unfavorable outcome. We present a case of a 4-year-old boy with progressive dyspnea due to tension pneumothorax. After chest tube insertion, the CT scan showed large multilocular cystic lesions containing air and solid areas involving the right lower lobe, and its related pleura. Thoracotomy was carried out, and the cyst was removed with the pleural solid areas. Histopathological examination confirmed the diagnosis of PPB type II. Postoperatively, the patient was scheduled to start chemotherapy in a specialized pediatric oncology center to complete the treatment.