Young adults with cystic fibrosis (CF) (n = 73) were found to have age-adequate psychological functioning. Psychological adjustment, as measured by the Psychological Screening Inventory (PSI) and the Tennessee Self-Concept Inventory (TSCI), correlated with coping style as measured by the Repression-Sensitization (R-S) scale. A repressive or avoidant coping style was associated with higher self-esteem, lower alienation and discomfort, and greater social activity, but not with employment status or school attendance. Severely ill patients were less likely to be employed or in school, and showed higher levels of psychological discomfort. Physicians' ratings of coping effectiveness were positively related to a repressive illness-related coping style. Prospective assessment of psychological functioning would yield useful information concerning the coping strategies used by young adults with CF to ameliorate the psychological distress associated with physical deterioration.