Euglycemic Diabetic Ketoacidosis Presenting as Hypoglycemia in a Patient With Type 2 Diabetes and Von Gierke's Disease.

Von Gierke's disease (VGD) is rarely associated with type 2 diabetes mellitus (DM2). We present a case of VGD with DM2 that presented with hypoglycemia in the setting of diabetic ketoacidosis (DKA) in a young male with VGD using the sodium-glucose co-transporter 2 (SGLT2) inhibitor empagliflozin. The patient required resuscitation with fluids containing glucose prior to the administration of insulin for DKA protocol with significant clinical improvement.