Pneumoblastoma is a rare malignant pulmonary tumor. A new case is reported in a 2 years 5 month-old girl. Because the lack of locoregional extension an isolated surgical excision was performed. However, 6 months later, a local relapse occurred, with inframediastinal extension and cerebral metastases. On the occasion of this case, the authors review the problems set by the histogenesis of this tumor, its therapeutic indications, poorly codified due to its rarity and, overall, its often unfavourable outcome.