Abstract Sjӧgren's syndrome (SS) is a systemic autoimmune disease affecting mainly the exocrine glands. Tubulointerstitial nephritis (TIN) is the most predominant renal involvement with lymphocyte and plasma cells infiltration of the interstitium. We report a case of young female with SSpresented withrenal tubular acidosis (RTA), polyradiculoneuropathy &proteinuria. Renal biopsy revealed granulomatous interstitial nephritis. Granuloma formation is rarely seen in cases of SS. Key Words:Sjӧgren's syndrome, Granulomatous tubulo-interstitial nephritis