Diabetes in tropical regions and in some developing countries presents clinically differently from IDDM and NIDDM in the Western world and developed countries. So, the WHO recognized malnutrition-related diabetes mellitus and subdivided into fibrcalculous pancreatic diabetes(FCPD) and protein-deficient pancreatic diabetes in 1995. While it appears that malnutrition may influence the expression of other types of diabetes, the evidence that diabetes can be directly caused by malnutrition is not convincing. Thus, at present time, FCPD is best considered as a secondary form of diabetes. FCPD is a form of diabetes with a high prevalence in tropical and developing countries, but rare in Korea. It occurrs in young indi.viduals, accompanied by pancreatic calculi and or ductal dilatation of unknown cause, and is ketosis-resistant. We experienced a case of FCPD with familial tendency and report with a brief review of the literature.