Sjögren's syndrome is a systemic autoimmune disorder that affects the exocrine glands and characterized by the development of lympho-plasma cell infiltrate, which causes progressive loss of glandular function. Primary form involves the exocrine glands, with or without systemic involvement; and also, a secondary form, which is associated with other autoimmune diseases e.g. rheumatoid arthritis, lupus erythematosus, scleroderma, inflammatory vascular and connective tissue diseases etc. Overt or latent Renal Tubular Acidosis (RTA) caused by tubule-interstitial nephropathy is a common extra-glandular manifestation of Primary Sjögren's Syndrome (PSS). PSS is most frequently diagnosed in female (female: male ratio of 9:1) and mostly diagnosed based on the results of salivary and/or lachrymal gland biopsies, examination of oral cavity and eyes and autoantibody assays. Here we present a case of 38 years old female patient with hypokalemic quadriparesis secondary to PSS.