Cerebral vasculitis represents a rare form of vascular inflammatory involvement caused by heterogeneous conditions. In this study, the wide spectrum of cerebral vasculitis despite primary angiitis of the CNS is analyzed. Our cohort included 16 white patients with cerebral vasculitis treated in a single German institution between 2003 and 2008. Clinical and diagnostic features were obtained by retrospective chart review; follow-up information and outcome were obtained prospectively. The spectrum of conditions responsible for cerebral vasculitis included seven patients with Behçet syndrome and one case each of giant cell arteritis, Wegener's granulomatosis and Churg-Strauss syndrome, respectively. Vasculitis secondary to systemic diseases included two patients with systemic lupus erythematodes, one with sarcoidosis and one with ANA-positive systemic vasculitis. Two patients suffered from infectious angiitis caused by borreliosis and syphilis. The mean age at onset of cerebral symptoms was 41.38 years. The most frequent clinical symptoms were headache, gait disturbances and unilateral numbness. None of the patients with Behçet syndrome experienced any ischemic event, which was a significant difference compared with the other patients (P = 0.011). Gadolinium-enhancing lesions were significantly more frequent in Behçet syndrome compared to the other types of vasculitis (P = 0.041). There was no significant difference between vasculitis patients with or without Behçet syndrome regarding outcome parameters. The differential diagnosis of conditions responsible for cerebral vasculitis includes a wide spectrum of diseases. Clinical features and the course of cerebral vasculitis are highly variable. The enigma of cerebral vasculitis will only be solved by implementing large, prospective, multicenter databases.