Pleuropulmonary bastoma (PPB) is a rare intrathoracic malignant neoplasm in children, differ from pulmonary blastoma in adults. PPB is usually aggressive and has wide-metastases at the time of diagnosis. The therapeutic medality of PPB is extensive surgical resection with neoadjuvant or adjuvant chemotherapy. We report a case of a cystic pieuropulmonary blastoma treated with surgical resection and adjuvant chemotherapy.