Background. Routine administration of vitamin A, recommended in CF patients, can help to prevent its deficiency. However, high vitamin A supplementation may lead to its excessive level and possible toxicity. Therefore, the aim of the present study was to assess the status of vitamin A and the determinants of its body resources in CF patients. Material and methods. In 196 CF patients aged from 4 months to 47 years, the following parameters were analysed: nutritional status (standardized body weight and height, serum albumin concentration) and clinical expression of disease (lung function − spirometry; biochemical markers of liver function − ALT, AST, GGT; respiratory tract colonization by Pseudomonas aeruginosa; diabetes; cirrhosis, non-cirrhotic liver disease; exocrine pancreatic function − fecal elastase-1 concentration; blood clotting -INR and vitamin A supplementation). Results. Median vitamin A concentration in the study group was 383.0 ng/ml (1