The Online Mendelian Inheritance in Man phenotype 614338, including congenital PNLIP deficiency, congenital pancreatic CLPS deficiency, and combined congenital PNLIP and CLPS deficiency, is a rare monoenzymatic form of exocrine pancreatic failure. The gene discussed is PNLIP; the disease is hyperinsulinemic hypoglycemia, familial, 4.