Primary Sjogren's syndrome (PSS) is a chronic autoimmune disease characterized by lymphoplasmacytic infiltration of the exocrine glands, which results in multiple organs damage. Renal injury affects 0.3-27.0% of PSS patients, and tubulointerstitial nephritis is the most frequent form of nephropathy in PSS. The present study reports on the case of a 32-year-old female with a 1-year history of elevated serum creatinine levels, and a 6-month history of mild pain in the waist and leg. Blood biochemistry tests indicated a creatinine level of 221.3 mu mol/l and estimated glomerular filtration rate of 24.6 ml/min/1.73 m(2) [Chronic Kidney Disease Epidemiology (CKD-EPI) formula]. Accordingly, the patient was diagnosed with stage IV chronic kidney disease. To clarify the underlying cause of the disease, a kidney biopsy was performed, which revealed tubular epithelial cells with multiple focal and lamellar atrophy (similar to 60%), as well as extensive renal interstitial fibrosis with scattered inflammatory cell infiltration. Based on these results, the patient was finally diagnosed with severe chronic interstitial nephritis, chronic kidney disease stage IV, PSS and anemia due to chronic kidney disease. The patient was treated with half-dose glucocorticoid (prednisone, 25 mg oral qd maintained up to 12 months). The patient's serum creatinine levels had decreased to 172.4 mu mol/l after 1 month and to 178.7 mu mol/l after 12 months. The present case concluded that young patients with chronic renal failure should first be assessed for rheumatic immune system diseases. PSS may involve several organs and the clinical manifestations may be varied. Although chronic renal failure is frequently the first manifestation of renal disorder due to PSS, it may be overlooked by clinicians. The present results suggest that further attention should be paid to determine the association between symptoms in the clinical setting.