Sjögren's syndrome is a chronic inflammatory disease characterized by the infiltration and progressive destruction of salivary and lacrimal glands. A common presentation involves the complaints of dry eyes and dry mouth, known as the “sicca complex.” Extra-glandular involvement is not uncommon, and is known to involve the lungs, vascular and peripheral nervous system, and kidney.1 The reported renal involvement ranges broadly from 2 – 67%,and is variably defined: the most commonly reported renal pathology is an a tubulo- interstitial nephritis resulting in tubular dysfunction and immune-mediated glomerular disease; however distal renal tubular acidosis (RTA) in particular can be present in 20-30% of cases.2,3 Although there have been case reports of patients with Sjögren's syndrome presenting with distal RTA during the course of the disease, to our knowledge, a diagnosis of Sjögren's syndrome made solely on the basis of renal manifestations without any overt physical findings of the disease has not been documented.