There is a growing patient population with adult congenital heart disease that needs specialized medical attention and careful long-term evaluation. Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a common late complication, and is associated with increased morbidity and mortality. There are no clear current guidelines for the treatment of PAH-CHD. There are few trials to date investigating PAH treatment specifically in this group of patients. However, the available data seems to demonstrate that with the advent of PAH-targeted therapies, the quality of life, exercise capacity, and outcomes in these patients is improving. In addition, PAH-targeted therapies may be useful in select patients for a combined medical-surgical approach to treatment. Here we discuss the epidemiology and pathophysiology of PAH-CHD, current therapies, and the data supporting their use, and how to evaluate feasibility of late surgical repair.