The evolution of lung transplantation has offered an additional treatment option for patients with a variety of end-stage lung diseases, including some with systemic disorders. Lymphangiomyomatosis (LAM) is a multisystem disease of premenopausal women. The lung is the most frequently involved vital organ, and respiratory failure is the most common case of death. A small number of patients with LAM have undergone lung transplantation. This report describes a case of recurrent LAM in the allograft after single lung transplantation and discusses the implications of this finding.