Diabetes of the exocrine pancreas (DEP) used to be called pancreatic diabetes, pancreatogenic diabetes or type 3c diabetes mellitus. Currently, the incidence of DEP is higher than that of type 1 diabetes mellitus. The pathogenesis and clinical manifestations of DEP are related to primary pancreatic diseases. In terms of management, we need to consider both pancreatic endocrine and exocrine functions, and comprehensively treat diabetes mellitus and primary pancreatic diseases. By now, there has been no guideline related to DEP; its diagnostic criteria, differentiation with type 2 diabetes mellitus, and selection of hypoglycemic programs are challenges in clinical practice. This article reviews the clinical studies related to DEP, and summarizes the evolution of its terminology, pathogenesis, clinical manifestations, complications, diagnosis, treatment and management.