Cystic fibrosis is the commonest autosomal recessive disorder in Europe. It can present late with atypical features.1 Mutations in a gene on chromosome 7 that regulates transmembrane conductance cause the disease.2 Seventy two per cent of patients with cystic fibrosis are homozygous or heterozygous for eight mutations of the gene.3 The primary defect is low permeability of cell membranes to chloride ions which affects fluid transport during secretion and absorption by epithelial cells.4 We report a case of late presentation of cystic fibrosis in a man with the (del)F508 and R117H mutations.