Background. Pulmonary blastoma is a rare malignancy treated primarily with surgery. Chemotherapy has proven effective in some cases, and has been used in adjuvant fashion in pediatric patients. Method. This is a case report of a 40 year-old woman treated with surgical resection, and because of the high risk of recurrence, adjuvant chemotherapy was also given. Results. At the time of this writing, the patient has been in continued remission since surgery in October, 1993. Conclusion. This rare entity is difficult to diagnose noninvasively. The limited number of case reports makes therapeutic decisions difficult. To the authors' knowledge, this case is the first reported adult treated with adjuvant chemotherapy. A central registry of cases would be helpful to physicians treating patients with this disease.