The association of either thrombotic thrombocytopenic purpura (TTP) or crescentic glomerulonephritis with Sjögren's syndrome is rare. We report a case of TTP appearing after the diagnosis of SjOgren's syndrome with crescentic glomerulonephritis and membranous nephropathy. Circulating immune complex was detected, and immune complex deposits were shown along the capillary walls of renal biopsy specimens. Despite steroid pulse therapy and plasma exchange therapy, the patient died. The etiology of TTP is unclear. This case is important when considering the etiology of TTP related to autoimmune disease.