Vasculitis is a complication happening in a third of patients with primary Sjögren's Syndrome. It can be of 2 types: neutrophilic or mononuclear and sometimes mixed. Very occasionally, a necrotizing vasculitis polyarteritis nodosa type during the evolution of Sjögren's Syndrome has appeared. A case of a female patient with a Sjögren's Syndrome of large evolution, who suddenly showed a poly-systemic affliction (nervous system, kidneys, muscles and digestive system) secondary to a necrotizing vasculitis type polyarteritis nodosa with a good response to immunosuppressors, is presented.