As increased experience is gained in the field of lung transplantation, novel applications for this life saving therapy will evolve. We have described a case of bilateral lung transplantation in a young patient with respiratory failure secondary to metastatic leiomyosarcoma that was limited to the lungs. While lung transplantation for malignancy remains a controversial area, this case illustrates that lung transplantation can provide improved quality and quantity of life for highly selected patients with malignant disease.