We present a case report of a patient with Sjögren's syndrome accompanied with interstitial cystitis. A 64-year-old woman complained of dry mouth for 21 years, recurrent swelling and pain on the right parotid in 2000, and urinary irritation symptoms in the past 2 years. Several courses of different types of antibiotics could not relieve her urinary irritation symptoms. Bladder hydraulic dilatation had only transient effects. Urine sediment contained neither erythrocytes nor leukocytes, and urine cultures yielded no growth. However, increased γ-globulin, positive antinuclear antibodies, and anti-SS-A and anti-SS-B antibodies were detected. Histopathological data of labial salivary glands showed three foci of numerous mononuclear cells. Cystoscopy revealed redness, edema, angiectasis, and extensive ecchymosis of the mucosal surface and several small floating cruor entities in the bladder. Urinary bladder biopsy specimens revealed the absence of urothelium and edematous lamina propria and submucosa, with diffuse or multiple focal chronic inflammatory cell infiltration. Based on these findings, she was diagnosed with Sjögren's syndrome accompanied with interstitial cystitis. Therapy with corticosteroids relieved the symptoms significantly.