Autoimmune diseases can affect the blood vessels, causing systemic vasculitis. Although testicular manifestation of some autoimmune diseases is not uncommon, only a few cases of acute orchitis are described in the literature. The underlying pathological condition in testicular manifestations of autoimmune diseases is severe vasculitis causing inflammation and infarction. In patients with recurrent episodes of scrotal swelling and pain, testicular vasculitis as the first sign of a systemic disease should be taken into consideration.