We aimed to examine the differences between patients with cystic fibrosis-related diabetes (CFRD), and those with normal glucose handling in adults with cystic fibrosis (CF) in Ireland. We conducted a retrospective analysis of patients who attend the national referral centre for adult CF. Patients were diagnosed as having CFRD by the American Cystic Fibrosis Foundation criteria for diagnosis of CFRD. Of 259 patients, 150 were classifiable and 81 (54%) were classified as having CFRD. The groups with and without CFRD were not significantly different with regard to age (median 28.4 vs 26.0 years), sex (males 56% vs 55%) or BMI (median 20.9 vs 21.3 kg/m2). The group with CFRD had poorer lung function (mean % predicted FEV1 49.9 vs 66.4, P < 0.001), poorer bone mineral density (T-scores at the lumbar spine -1.95 vs -1.44, P < 0.05 and femur -1.19 vs -0.57, P < 0.01) and a greater proportion of PSEUDOMONAS AERUGINOSA positive sputum cultures (82.5% vs 64.2%, P < 0.05). No patients with CFRD carried the R1 17H mutation whilst 19% of the group without CFRD were heterozygous for this defect (P < 0.001). In conclusion, CFRD was highly prevalent in adults. The presence of CFRD was associated with poorer lung function, poorer bone mineral density and an increased prevalence of PSEUDOMONAS AERUGINOSA in sputum. The R1 17H mutation may be protective for CFRD.