Cystic fibrosis is characterized by dysfunction of lungs, pancreas, skin and other organs. In some ethnic groups, it is the most common potentially lethal autosomal recessive disorder. In countries where neonatal screening for cystic fibrosis is routine, clinical problems can be anticipated and largely avoided. In other countries, one initial presentation of the disease may be in infancy with failure to thrive, oedema and well-demarcated, erythematous, scaly plaques covering most of the body. This presentation is related to kwashiorkor secondary to malabsorption and responds to pancreatic enzyme replacement. In older children, aquagenic wrinkling of palms can be seen in some children with cystic fibrosis and also in normal children. With current management protocols for cystic fibrosis, the life expectancy is now expected to be greater than 50 years.