The aim of this review is to describe recent developments in the field of pancreatic disorders in children. First, recent developments in the genetic research of hereditary pancreatitis are discussed. Subsequently, several issues of acute pancreatitis are presented. These include a description of the potential hazardous morbidity and mortality of this disorder in children. In addition, various novel etiologies that have been described lately in the medical literature are illustrated. Next, this paper discusses two examples of pancreatic disorders associated with systemic manifestations, i.e., ganglioneuromatosis and diabetes mellitus. Finally, a few rare genetic syndromes with pancreatic involvement are touched upon, an association that is not very well recognized. Cystic fibrosis is not covered.