Abstract: Eisenmenger’s syndrome is a kind of pathophysiologic syndrome that occurs in the end stage of congenital heart disease. The diagnosis of Eisenmenger’s syndrome is clear, but its early clinical presentations are nonspecific and its prognosis is poor. Recently, the therapeutic regimen is steadily improved by the endless efforts that lots of scientists have made. Treatments including new drugs, modified surgery, extracorporeal membrane oxygenation, transplantation and gene therapy can not only relieve symptoms, correct abnormal pulmonary hemodynamics, but also can improve prognosis to some extent. We will review and introduce the advance of research on Eisenmenger’s syndrome, including its pathogenesis, diagnosis and treatment.