GSDs are classified according to the specific enzyme deficiency and the affected organ and include GSD 0a, GSD 0b, GSD I, GSD II (Pompe disease, PD), GSD III, GSD IV, GSD V, GSD VI, GSD VII, GSD IX, GSD X, GSD XI (lactate dehydrogenase subunit A deficiency), GSD XII, GSD XIII, phosphogluco-mutase 1 deficiency–congenital disorder of glycosylation (PGM1-CDG; previously called GSD XIV), GSD XV, Fanconi–Bickel syndrome (FBS), and phosphoglycerate kinase (PGK) deficiency [1,4]. Here, PRKG1 is linked to hyperinsulinemic hypoglycemia, familial, 4.