The life span of patients with cystic fibrosis (CF) continues to extend due to advances in treatments and care. The rate of pregnancy for female patients with CF has also continued to rise. The purpose of this study was to systematically review the available literature on female patients with CF and their knowledge of sexual and reproductive health. A priori, the research question was posed: What deficits in sexual and reproductive health knowledge exist among women with cystic fibrosis? Three broad areas of knowledge lacking in female patients with CF were identified: physiological, genetic, and psychosocial. The results imply female patients with CF are not equipped with adequate information for the decision-making process involved in sexual and reproductive choices. Social workers play an important and dynamic role on the multidisciplinary CF care team and can improve the quality of life for women with CF by addressing the psychosocial processes associated with the disease. Because of social work's important role in the holistic care approach to CF and other chronic and debilitating illnesses, social work literature should focus greater attention on these issues.