This article reviews 31 recent articles that describe the functioning of families and/or parents of children with cystic fibrosis (CF), compares them to families of healthy children, pinpoints factors that predict quality of adjustment, or evaluates interventions designed to improve functioning. Commonly cited concerns included the difficulty of the treatment regimen, the terminal nature of CF, and the disruption of intrafamilial relationships. Parents of children with CF experienced greater stress and burdens than parents of healthy children, yet parenting behavior and family functioning were quite similar in CF and healthy control groups. Higher levels of distress, an avoidant coping style, and low levels of family support were associated with poor psychological adjustment. Recommendations for future research include: using multiple informants; using detailed, illness-specific measures and conceptual frameworks; and conducting studies of individual variation and interventions. Practitioners might consider performing periodic updates of how the family is managing as a whole.