We report a 25-year-old woman presenting with a flaccid paresis due to severe hypokalaemia as a consequence of distal renal tubular acidosis (dRTA). Six years after pre-sentation of dRTA, she developed overt symptoms of sys-temic lupus erythematosus (SLE). dRTA in SLE is often sec-ondary to an interstitial nephritis. In contrast to other reports the dRTA did not resolve after treatment with prednisone in our patient. Nephrocalcinosis might be one of the causal fac-tors in the persistence of dRTA.