This critical review of the literature on cystic fibrosis (CF) emerged as the result of the work of a national group that was convened to understand and advance the state of research on families and chronic illness. Several family scientists reviewed empirical studies regarding families and specific chronic diseases affecting children, adults, and the elderly. The authors report the substantive empirical findings regarding families who have children with CF. A total of 54 studies, published in the last 2 decades, are reviewed and critiqued. Strengths and limitations of these studies are discussed as well as conclusions and implications for theory and research in the area of families and childhood chronic illness.