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Diabetic ketoacidosis is the most common cause of death in children who have type 1 diabetes. Severe diabetic ketoacidosis is a grave illness and is the commonest cause of diabetes-related deaths in children and adolescents.  The object of this review is to provide the definitions, pathophysiology, frequency and risk factors and management recommendation for diabetic ketoacidosis in children. Deaths should be avoidable by reducing the incidence of diabetic ketoacidosis by earlier diagnosis at onset, immediate referral and urgent treatment, appropriate management of diabetes and optimal management of diabetic ketoacidosis