The term renal tubular acidosis (RTA) is applied to a group of transport defects in the reabsorption of bicarbonate (HCO3-), the excretion of hydrogen ions, or both. On clinical and pathophysiological grounds, RTA can be separated into three main types: distal RTA (type 1), proximal RTA (type 2) and hyperkalaemic RTA (type 4). Some patients present combined types of proximal and distal RTA or of hyperkalaemic and distal RTA. Diagnosis of RTA should be suspected when a patient presents a normal plasma anion gap, and hyperchloraemic metabolic acidosis. A normal plasma anion gap (Na(+)-[Cl- + HCO3-] = 8-16 mEq/l) reflects loss of HCO3- from the extracellular fluid via the gastro-intestinal tract or the kidney, dilution of extracellular buffer or administration of hydrochloric acid (HCl) or its precursors. Distinction of RTA from other disorders is greatly facilitated by the study of the urine anion gap (Na+ + K+ - Cl-). This index estimates the urinary concentration of ammonium in a patient with hyperchloraemic metabolic acidosis. A negative urine anion gap (Cl- much greater than Na+ + K+) suggests the presence of gastro-intestinal or renal loss of HCO3-, while a positive urine anion gap (Cl- less than Na+ + K+) is indicative of a distal acidification defect. Determination of plasma potassium, of urine pH at low plasma HCO3- concentration, and of urine PCO2 and fractional excretion of HCO3- at normal plasma HCO3- concentration permits the differentiation between the various types of RTA.