Diabetes is a common complication of cystic fibrosis (CF), occurring in approximately 40% of adult patients. It is clinically distinct from type 1 or type 2 diabetes, and requires a unique approach. Because of evidence that it is associated with increased morbidity and mortality, prompt diagnosis and aggressive management of CF-related diabetes (CFRD) is important. The Cystic Fibrosis Foundation held a consensus conference in 1998 to define the current standards for the diagnosis and care of this disease. This article reviews those recommendations, and presents a practical approach to the management of CFRD.