ANCA-associated vasculitis is a rare disease characterized by inflammation of small vessels in organs such as the kidneys, lungs, upper respiratory tract, skin, eyes, and peripheral nerves, with necrotizing crescentic glomerulonephritis as the typical manifestation in the kidneys. Sjögren's syndrome is an autoimmune disorder with symptoms of dry eyes and dry mouth. The article describes a case of overlapping of these two diseases in an elderly male patient. The patient showed significant improvement after treatment with cyclophosphamide, glucocorticoids, hemodialysis and rituximab. A review of the literature identified 49 cases of ANCA vasculitis overlapping with Sjögren's syndrome, which were summarized and analyzed.