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Purpose of Review: This article provides an overview of endocrine emergencies with potentially devastating neurologic manifestations that may be fatal if left untreated. Pituitary apoplexy, adrenal crisis, myxedema coma, thyroid storm, acute hypercalcemia and hypocalcemia, hyperglycemic emergencies (diabetic ketoacidosis and hyperglycemic hyperosmolar state), and acute hypoglycemia are discussed, with an emphasis on identifying the signs and symptoms as well as diagnosing and managing these clinical entities.Recent Findings: To identify the optimal management of endocrine emergencies, using formal clinical diagnostic criteria and grading scales such as those recently proposed for pituitary apoplexy will be beneficial in future prospective studies. A 2015 prospective study in patients with adrenal insufficiency found a significant number of adrenal crisis–related deaths despite all study patients receiving standard care and being educated on crisis prevention strategies, highlighting that current prevention strategies and medical management remain suboptimal.Summary: Early diagnosis and prompt treatment of endocrine emergencies are essential but remain challenging because of a lack of objective diagnostic tools. The optimal management is also unclear as prospective and randomized studies are lacking. Additional research is needed for these clinical syndromes that can be fatal despite intensive medical intervention.