Women with cystic fibrosis (CF) are living to childbearing age and many have successful pregnancies. Preconception care with optimization of pulmonary function, eradication of pulmonary infection, improved nutritional status, and diabetes care improve fertility and pregnancy outcome. Women with CF, poor pulmonary function and nutrition, and less than ideal body weight are more likely to suffer adverse outcomes. Women with CF and pulmonary hypertension risk mortality. Individuals with CF and end stage lung disease have improved survival after lung transplant. Women with lung transplants can have successful pregnancy, but the risk of organ rejection and death are high.