The pancreas is an important exocrine and endocrine organ that develops from the dorsal and ventral anlagen during embryogenesis and arises from the endodermal lining of the duodenum within the first month of human embryonic life. A number of developmental disorders can either lead to anatomic abnormalities of the pancreas and its ducts, or can be part of complex disorders that affect multiorgan systems. Other genetic changes can lead to metabolic abnormalities that affect the pancreas exclusively or increase the lifetime risk for developing pancreatitis or pancreatic diabetes. This article reviews some of the developmental and metabolic disorders that can affect the endocrine and exocrine pancreas.