The primary systemic vasculitides are a rare group of inflammatory disorders, where damage is directed against the blood vessels. They range in severity from acute self-limiting illness to chronic life and organ threatening diseases requiring long-term treatment with toxic immunosuppressive regimes. None of them have specific tests and few have hard diagnostic criteria. This review summarises the gastrointestinal features of these diseases, along with their general clinical and epidemiological features and the evidence available to guide in their management.