In January 1998, a 14-month-old girl presented with fever and dyspnoea. Plain chest radiograph showed opacification of the right hemithorax that corresponded to a multicystic mass on computed tomography. Thoracotomy was performed and a 11-cm × 9.5-cm × 5-cm mixed cystic-solid mass excised. Together with histological findings, type II pleuropulmonary blastoma (PPB) was diagnosed. The patient was treated with adjuvant chemotherapy according to the Intergroup Rhabdomyosarcoma Study-IV regimen but developed ifosfamideinduced renal tubulopathy. At the time of writing, the patient remains in remission (age 23 years). Sanger sequencing revealed no DICER1 mutation in the patient's peripheral blood DNA.