Mitral valve prolapse is a cardiac condition in which the mitral valve is incompetent. With the use of sex-specific risk factors and risk markers, it is possible to detect the severity of a mitral valve prolapse in pregnant women with congenital heart disease. Withal, in many cases these women are not aware of having cardiac problems and therefore it is crucial to create awareness amongst them and also amongst physicians. During pregnancy, there is an increase in cardiac output that could possibly worsens pre-existing cardiac conditions. Most of these patients can tolerate their pregnancy well if they receive an adequate medical control. However, pregnancy should be avoided when there is a high chance of developing mitral valve prolapse. Finally, the necessity of future research to identify underlying mechanisms influencing the risk factors and risk markers of cardiovascular disease in women will be discussed.