Euglycemic diabetic ketoacidosis (EDKA) is a rare, acute, life-threatening emergency that is characterized by euglycemia, metabolic acidosis, and ketoacidosis. Unlike DKA, the diagnosis of EDKA is often overlooked because of the absence of hyperglycemia. The mechanism behind EDKA involves a general state of starvation that results in ketosis while normoglycemia is maintained. EDKA may be associated with precipitating factors, including sodium-glucose cotransporter 2 inhibitors, starvation, pregnancy, alcohol use, surgery, and drug-induced intoxication. A stepwise approach is used in the management of EDKA. Pharmacists can assist the medical team in preventing EDKA and can play a role in the management of EDKA.