Type 4 RTA is caused by a defect in the distal tubule, but it is different from classic distal RTA and proximal RTA because it results in hyperkalaemia rather than hypokalaemia. RTA type 4 is in effect hyporeninaemic hypoaldosteronism.

It occurs when blood levels of aldosterone are low or when the kidneys do not respond to it. Fludrocortisone is usually effective as a form of treatment.

Type IV RTA may result from may result from sickle cell disease, urinary tract obstruction, lupus, amyloidosis, or transplantation