As the life span of patients with cystic fibrosis has increased, so has the prevalence of cystic fibrosis-related diabetes mellitus. However, screening practices for cystic fibrosis-related diabetes mellitus vary widely, which affects accurate estimates of the health burden of this comorbidity. The management of prediabetes and hyperglycemia is an increasingly important aspect of care in patients with cystic fibrosis, but few studies have specifically addressed the management of cystic fibrosis-related diabetes mellitus. Previous studies support the use of insulin for the treatment of patients with this disorder, but the evidence for its use in patients with cystic fibrosis and impaired glucose tolerance is poor. Nutritional management is currently guided by dietary recommendations for individuals with cystic fibrosis, with little evidence specific to the dietary management of patients with cystic fibrosis-related diabetes mellitus. Additionally, microvascular complications have become more frequent as a result of the rise in life expectancy of these patients, yet to date no intervention studies have addressed prevention or management of diabetic complications in patients with cystic fibrosis. A strong evidence base is needed to guide the management of patients with cystic fibrosis-related diabetes mellitus and its complications.