Pulmonary blastoma (PB) is the least common malignant pulmonary neoplasm, with less than 100 reported cases. This tumour occurs up to three times more commonly in males. There exists an age peak in the third and fourth decades, although approximately one quarter of cases occur in children, often in association with congenital lung disease. Although the clinical features of PB are not specific, the histopathological appearance is distinctive, showing an admixture of both epithelial and mesenchymal (sarcomatous) elements. Historically, surgery has been the most commonly used modality for localized disease: however, useful data on local control rates are lacking. Extrathoracic metastases are the major cause of treatment failure in PB, with chemotherapy having little impact in this disease. The role of radiation therapy in the management of PB has not been established, however, early radioresponsiveness was demonstrated in the palliative treatment of soft tissue and bone metastases in the case presented: radiation therapy should be considered in the management of this disease.