Primary arteritis of the central nervous system (CNS) comprises a heterogeneous group of CNS disorders, which is characterised by non-atheromatous inflammation and necrosis of the arterial wall. The clinical presentation is highly variable, with stroke being the most common manifestation. Conventional angiography is considered to be the best imaging tool for diagnosing the disease. However, angiographic findings, which usually show lumen irregularities and stenosis, are often unspecific and can occur with a variety of other vascular disorders, such as atherosclerosis and arterial dissection. Therefore, brain biopsies are often needed to confirm the diagnosis. Recent reports have shown that MRI is able to visualise contrast enhancement in subjects with known primary CNS arteritis.