Abstract
Hypoglycemia, especially when recurrent or persistent, is an important indicator of inborn metabolic errors. Although commonly encountered by hepatologists, it continues to be a pandora's box as no consensus on the exact definition and diagnostic work up exists. Here, we present four interesting pediatric cases of varied age groups, presenting with hypoglycemia as their major symptomatology. We also attempted to provide a systematic diagnostic guide for a refined and targeted approach to inherited metabolic liver diseases presenting with hypoglycemia.