Remarkable advances in surgical and clinical management have resulted in survival to adulthood in the large majority of patients with congenital heart malformations, even with the most complex disease. Over 1 million adults with congenital heart disease currently live in the USA, approximately half of whom are women of childbearing age. Collectively, congenital malformations are the most common form of heart disease in pregnant women. Indeed, in North America, congenital defects are now the leading cause of maternal morbidity and mortality from heart disease. This article begins with a summary of cardiovascular changes during pregnancy and highlights key features in pre-pregnancy counseling, maternal cardiac and obstetric risk, and neonatal complications. Management issues regarding pregnancy and delivery are elaborated, including anesthesia considerations. While it is beyond the scope of this article to discuss particulars related to all forms of congenital heart disease, selected subtypes are detailed at greater length. In the absence of clinical trial evidence to inform the care of pregnant women with congenital heart disease, this article is inspired by the premise that knowledgeable multidisciplinary assessment and management provides the best opportunity to substantially improve outcomes for mother and baby.