We performed emergency living-donor lobar lung transplantation (LLTx) successfully in a 24-year-old woman with end-stage pulmonary and massive abdominopelvic lymphangioleiomyomatosis (LAM). Preoperatively, her respiratory condition was critical, but abdominopelvic lesions had been well controlled with medication. No LAM patients with massive abdominopelvic lesions as in the present case have been reportedly treated by LLTx previously. The present case demonstrates that LLTx can be a therapeutic option for end-stage pulmonary LAM with massive abdominopelvic involvement.