Systemic lupus erythematosus (SLE) is a multisystem disease with marked variability in its manifestation. Tuburlointerstitial involvement is well recognized in SLE. But usually the tubular dysfunction is latent and usually presents after diagnosis of SLE. We report a 20 years old female whose initial symptom of SLE was distal renal tubular acidosis (RTA). She presented with severe muscle weakness at emergency room with laboratory finding consistent with distal RTA. After several months she developed fever, arthritis, serologic finding which was compatible to diagnose SLE. We report a case whose initial symptom of SLE had been distal RTA.