Pulmonary blastoma is a rare primary lung neoplasm, occurring in both children and adults, which is pathologically, clinically, and prognostically distinct from other lung tumors. Usually it has been treated with surgery, but both chemotherapy and radiotherapy have been used for metastatic disease, and in the adjuvant setting. A patient is described who presented with metastatic pulmonary blastoma. Treatment with cyclophosphamide, vincristine, doxorubicin, and dactinomycin resulted in an objective response as judged by standard criteria. The literature is reviewed for other experience with chemotherapy in this rare lung tumor. This four-drug combination appears to show promise for tumor response, and is deserving of further trial.