Cystic fibrosis (CF) is an autosomal recessive disorder caused by pathologic mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene located on chromosome 7. Patients with CF are at risk for dysglycemia which ultimately leads to cystic fibrosis-related diabetes (CFRD). As medical advances continue to extend the lifespan of people with cystic fibrosis, comorbidities like CFRD that used to affect only a segment of the aging population are beginning to be more common. Our continued understanding of the prevalence, screening guidelines, mechanisms, and treatment goals of CFRD for patients are critical to providing optimal care to the CF community. In this chapter, we will explore a detailed overview of CFRD as well as specific nutritional guidelines, treatment regimens, uses of diabetes technology in the CF population, as well as how new modulator therapy may play a role in disease progression and treatment.