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quent, but it cannot be dismissed. Early recognition along with prompt and appropriate medical and obstetrical management is critical. Genetic variants in hepatic nuclear factor 1α ( ) cause maturity-onset diabetes of the young (MODY). We sought to examine whether MODY variants also cause neonatal hypoglycemia. We present 3 infants with variants in shared with their mothers. The infants experienced neonatal hypoglycemia, 2 extending beyond 1 year and the third resolving by 28 days, and all were large for gestational age (birth weights of