Objective: To perform a systematic review of the literature to examine original research on contraception in women with cystic fibrosis (CF) with a specific focus on safety, efficacy, non-contraceptive benefits, and utilization patterns in this population. Study design: We searched PubMed and Embase databases for any peer-reviewed original research in English on the use of contraception in women with CF. Results: We identified 241 unique citations. After title review and exclusion of articles not reporting original data, 18 publications were included in the final review, with at least two articles for each area of focus. Limited evidence indicates that oral contraceptive pills are likely safe and efficacious in women with CF. Hormonal contraception may reduce pulmonary exacerbation rates and decrease need for antibiotics, suggesting non-contraceptive benefits. Women with CF utilize birth control at similar rates as the general population, and oral contraceptive pills and condoms are the most commonly used methods. Conclusions: Data on the safety, efficacy, and non-contraceptive benefits of hormonal contraception in women with CF are scant. Based on the limited data, hormonal contraception seems to be safe and efficacious and may provide noncontraceptive benefits. Further high-quality data from disease-specific research are required to better inform contraceptive decision-making among women with CF. (C) 2016 Elsevier Inc. All rights reserved.