2-year-old girl was diagnosed with pleuropulmonary blastoma (PPB). She presented with cough and fever which was treated as pneumonia. When her symptoms did not improve, a chest X-ray showed a right lower lobe infiltrate with pleural effusion and a cystic lesion superiorly. She was further treated with antibiotics and improved clinically, but follow-up with chest CT showed a persistent cystic area in the right lower lobe and pneumothorax. She thus underwent pleural drainage and thoracoscopic biopsy, revealing a diagnosis of "cystic pleuropulmonary blastoma".

2-year-old female had been diagnosed with Pleuropulmonary Blastoma. The disease extent included the lung and pleura with no signs of metastatic spread. It was decided to start with 3 cycles of chemotherapy and afterwards to carry out a CT scan reassessment and surgical operation. The expert agrees that patients treated with combination of chemotherapy and complete surgical resection do better. Achieving total resection of the tumor results in a significantly better prognosis, whereas extrapulmonary involvement at diagnosis results in worse prognosis.