Lung transplantation is an established procedure to treat patients with end-stage lung disease. The criteria for recipient selection are broadening to include patients with congenital defects of the immune system, such as X-linked hypogammaglobulinemia (XLA). We report 2 cases of successful double lung transplantation in patients with XLA. The 2 men had developed bronchiectasis and end-stage lung disease despite early institution of intravenous immunoglobulin (IVIG) replacement therapy. Before transplantation, hypogammaglobulinemia was well controlled with IVIG in both patients. After transplantation, IVIG was administered every 48 hours during the first 10 days and then tapered slowly in the following weeks until returning to an every 3 weeks schedule. One patient has been followed up for 12 months and the other for 6 months. Lung function normalized in the first case and showed a restrictive pattern in the second one. Lung transplantation may be considered as a therapeutic option for patients with XLA and end-stage lung disease. Regular administration of IVIG overcomes the high risk of infections due to the severe immunodeficiency and the intensive immunosuppressive therapy.