Diabetes is a common complication of cystic fibrosis. Because it is associated with increased morbidity and mortality, prompt diagnosis and aggressive management of cystic fibrosis-related diabetes is important. The Cystic Fibrosis Foundation held a consensus conference in 1998 to define the standards of care for patients with this disease [1[symbol: see text]]. In this article, pathophysiology and management of cystic fibrosis-related diabetes are reviewed.