Central nervous system (CNS) vasculitis is classified into two categories based on whether it is primary or secondary. Primary CNS vasculitis is rare disorder of unknown cause that is restricted to the brain and spinal cord. Currently, there are no randomized clinical trials for treatment of primary CNS vasculitis. Therefore, treatment regimens for primary CNS vasculitis have been derived from therapeutic strategies used in other kinds of vasculitis. Early detection is important because corticosteroid treatment with or without cyclophosphamide can often prevent serious outcomes and may result in a favorable response. However, because some patients are intolerant or respond poorly to combination therapy, development of novel treatment options is eagerly awaited. Several immunosuppressive agents such as mycophenolate mofetil, tumor necrosis factor-α blockers, and rituximab could be options for refractory patients.