Lung transplantation has been performed successfully since 1983 in patients with various end-stage lung diseases including primary pulmonary hypertension. More than 10,000 lung transplants have been reported in The Registry of the International Society for Heart and Lung Transplantation. In contrast, a transplant law became effective in Japan only recently and 11 lung transplants have been performed with excellent results. We performed the first successful living-donor lobar lung transplantation for a 19-y-o-f with primary pulmonary hypertension on January 5, 2001 using her father's right lower lobe and her mother's left lower lobe. When the patients with primary pulmonary hypertension do not respond to prostacyclin therapy, lung transplantation is a workable option.