A 4-year-old boy had a pulmonary blastoma removed from the site of a persistent pulmonary cystic lesion present since 6 weeks of age. Bone metastasis appeared 4 months after diagnosis. Following radiation therapy to the bone and after 15 months of VAC chemotherapy, he developed further metastases which showed little response to adriamycin or radiation therapy. He died 2 years and 2 months after the initial operation. A review of other pulmonary blastomas associated with cystic lesions in children is presented.