Eisenmenger syndrome is characterized by elevated pulmonary vascular resistance and right-to-left shunting of blood through a systemic to pulmonary circulation connection. Treatment requires either lung transplantation with intracardiac repair or heart-lung transplantation. There are inadequate data regarding treatment alternatives when the patient is not a candidate for surgery. In this article, we report on the case of a 68-year-old woman with Eisenmenger syndrome related to congenital heart disease who was treated with inhaled iloprost and oral sildenafil for 2 years. Copyright © 2005 by Lippincott Williams & Wilkins.