ZNF808 is a primate-specific transcriptional repressor essential for human pancreatic development 1. As a KRAB zinc finger protein, ZNF808 primarily targets long terminal repeats of primate-specific MER11 transposable elements, silencing them through establishment of H3K9me3-mediated heterochromatin during early pancreas differentiation 1. This repression prevents aberrant activation of liver-identity genes that would otherwise disrupt pancreatic fate specification 1. Biallelic loss-of-function ZNF808 variants cause neonatal diabetes with highly variable phenotypes ranging from permanent neonatal diabetes mellitus with pancreatic agenesis to adult-onset diabetes 2. Clinical presentations include insulin-dependent diabetes diagnosed before six months of age, intrauterine growth restriction, and variable exocrine pancreatic insufficiency 23. Disease severity appears independent of exocrine pancreatic involvement, with some patients not requiring insulin treatment and maintaining normal exocrine function 2. ZNF808-associated diabetes predominantly occurs in consanguineous families with autosomal recessive inheritance 2. Recent case reports document novel associations with skeletal anomalies, though mechanistic relationships remain unclear 3. ZNF808 gene testing is recommended in consanguineous populations and individuals with monogenic diabetes of variable ages of onset 2.