Pulmonary lymphangioleiomyomatosis is a disease principally affecting women during child-bearing years that eventually leads to respiratory failure. Recently, it has been listed as an indication for lung transplantation. To date, no cases of recurrent lymphangioleiomyomatosis after lung transplantation have been reported, unlike the experience with sarcoidosis and giant cell interstitial pneumonia. At the University of Pittsburgh Medical Center, four patients have undergone single-lung transplantation for lymphangioleiomyomatosis. We now report that one of these cases developed recurrent lymphangioleiomyomatosis in the allograft lung.