Recently, germline truncating mutations in the microRNA-processing protein DICER1 gene have been reported in patients with pleuropulmonary blastoma (PPB) or the related familiar DICER1 syndrome which includes, besides PPB, cystic nephroma, Sertoli-Leydig cell tumors, medulloepitheliomas and embryonal rhabdomyosarcomas6. Here, DICER1 is linked to pleuropulmonary blastoma.