Background : Pleuropulmonary blastoma ( PPB) is a rare primary intrathoracic mesenchymal malignancy that occurs exclusively in early childhood. This report describes our institutional experience of 6 patients diagnosed with PPB over a 10-year period. Methods : We identified 6 patients diagnosed with PPB at Asan Medical Center Children’s Hospital from 2003 to 2013. Clinical features, pathology, treatment, and outcome were evaluated by retrospective medical record review. Results : Median age at diagnosis was 30.9 months (range: 16.9-47.4 months). Female to male ratio was 2:3. All 6 patients came to the hospital for intractable upper respiratory infection. Of them, two patients presented with mild dyspnea, one with severe dypnea, one with chest pain, and the other two with only persistent cough. Pathologic examination showed type II PPB in 5 and type III in 1. Two patients are cousins and one of them had TP53 gene mutation. One patient, whose initial CT scan suggested with congenital cystic adenomatoid malformation (CCAM), underwent upfront surgical resection. The other 5 patients had USG-guided needle biopsy followed by neoadjuvant chemotherapy (median 3.4 cycles) and received complete tumor resection. All received adjuvant chemotherapy after tumor resection (median 8.5 cycles): 1 with ICE (ifosphamide + cyclophosphamide + etoposide), 4 with IVADo (ifosphamide + doxorubicin + vincristine + dactinomycin), and 1 with VAC (vincristine + dactinomycin + cyclophosphamide). None received radiotherapy. Brain metastasis was not found in any patient. During median 36.6 months of follow up, all patients have survived in remission. Conclusion : Our experiences show that high index of suspicion for PPB is needed in early childhood patients with persistent upper respiratory symptoms. and current treatment strategy (neoadjuvant chemotherapy + tumor resection + adjuvant chemotherapy without radiotherapy) is effective for cure. A larger, multi-center study is required to confirm this and to define the optimal treatment strategy.