Nutritional disorders and delayed physiological maturation are commonly seen in patients with cystic fibrosis. In our consultation work with a pediatric pulmonary service, we identified a series of 13 adolescents with cystic fibrosis and an atypical eating disorder. They did not fully meet the diagnostic criteria for anorexia nervosa, but they demonstrated marked weight loss, food avoidance, amenorrhea, and body image and bodily function distortions in the face of either normal gastrointestinal function or adequate pancreatic enzyme replacement therapy. They also demonstrated significant depressive symptomatology associated with their eating disorder. We review the presenting characteristics of these patients and the case histories of two adolescent girls with this syndrome. The psychosocial literature on cystic fibrosis is briefly reviewed with a focus on factors associated with cystic fibrosis that may predispose patients to the development of eating disorders. The authors also hypothesize that adolescents with other chronic illnesses may likewise be vulnerable to the development of eating disorders.