Cardiac disease is a leading cause of morbidity and mortality in pregnant women. An increased prevalence of the cardiovascular disease has been found in women of childbearing age, in which the responsibility of the treating physician extends to the mother and to the unborn fetus. As a result, care of these high-risk pregnant women with cardiovascular disease including those with congenital heart disease (CHD) require a team approach including specialists in maternal-fetal medicine, adult congenital cardiology, and obstetrical anesthesia. The human body undergoes significant amounts of physiological changes during this period of time and the underlying cardiac disease can affect both the mother and the fetus. Today, most female children born with CHD will reach childbearing age. For many women with complex CHD, carrying a pregnancy has a moderate to high risk for both the mother and her fetus. This chapter will review the epidemiology, risk factors, clinical presentation including common signs and symptoms, physiological changes in pregnancy, and the medical approach including cardiac medications, percutaneous interventions, and surgical procedures for pregnant women with CHD.