The authors report on four children, who were treated for a very rare pulmonary neoplasm, pleuropulmonary blastoma (PPB). This disease has been defined recently. The definition includes pulmonary blastoma, embryonic sarcoma, pulmonary rhabdomyosarcoma, pulmonary sarcoma, embryonic sarcoma and malignant mesenchymoma. PPB is histologically close to primitive mesodermal origin of the lung. Pleuropulmonary blastoma is a highly aggressive tumour, that exhibits unique histological, biological and clinical behaviour. The authors describe diagnostic and therapeutic steps, histopathological classification and treatment outcome. Two of four patients survive without any signs of malignant disease 9 and 14 years after treatment. One patient succumbed to disease progression and one to toxic complications related to treatment. The authors wish to inform pediatricians about this disease and make them consider pleuropulmonary blastoma in their differential diagnosis.

        Key words: pneumoblastoma, children, therapy