Hypovitaminosis A is associated with exocrine pancreatic insufficiency in cystic fibrosis. Peripheral retinal dysfunction is an early finding of vitamin A deficiency. We evaluated serum retinol and zinc as well as visual adaptation in 41 patients with cystic fibrosis, receiving generous pancreatic enzyme and micronutrient supplementation. Forty-one normal individuals matched for age and sex served as controls. Peripheral retinal function was measured by clinical electroretinography using an Electrophysiologic Personal Interfaced Computer and applying a standard protocol. Serum retinol in cystic fibrosis was significantly lower than that of the control group (0.30+/-0.01 versus 0.39+/-0.02 mg/l, p<0.001). Serum zinc concentrations were normal in the cystic fibrosis group (1.21+/-0.03 mg/l) and significantly higher than that of the control group (1.02+/-0.01 mg/l, p<0.001). The overall visual adaptation, however, was found to be normal and comparable in the two groups. It is concluded that, in cystic fibrosis, despite appropriate vitamin A supplementation, retinol serum concentration may be low. As serum retinol does not reflect vitamin A status, evaluation of visual adaptation may be a more appropriate way to monitor for vitamin A deficiency in cystic fibrosis.