Diabetic ketoacidosis (DKA) is a potentially life-threatening metabolic emergency with a high mortality rate. DKA is characterized by a diagnostic triad of hyperglycaemia, ketosis (ketonemia, ketonuria) and metabolic acidosis. Pathophysiologically, DKA results from an absolute or relative insulin deficit leading to an uninhibited lipolysis and thereby ongoing ketogenesis. Although patients with type 1 diabetes mellitus are most likely to be affected by DKA, this condition is increasingly found in those with type 2 diabetes. Most often, DKA is caused by lapsus in insulin therapy or (febrile) infections. Correct diagnosis of the metabolic disorder without delay is crucial for the prognosis. Therapy of DKA is based on fluid replacement, insulin therapy (initially intravenous application), and potassium repletion to prevent hypokalaemia. Differential diagnosis includes hyperglycaemic hyperosmolar syndrome (HHS), which is characterized by a relative insulin deficit; however, the leading symptom here is dehydration due to osmotic diuresis caused by severe hyperglycaemia. Thus, volume repletion is the most important step in therapeutic approach. For both DKA and HHS, it is essential to identify and eliminate the precipitating cause in order to prevent further episodes of metabolic decompensation. Both patients and doctors have to be familiar with this metabolic disorder to enable an adequate treatment and prevention of DKA.