The absence of G6Pase activity causes an impairment in endogenous glucose production resulting in lack of increase of blood glucose levels in response to positive gluco-regulatory stimuli; as a consequence, this condition is characterized by fasting hypoglycemia, high glycogen depots within liver and kidney, as well as lactic acidosis, hypertriglyceridemia and hyperuricemia as a consequence of shunting of G6P into alternative metabolic pathways [15,26,27,28]. This evidence concerns the gene G6PC1 and Hypoglycemia.