Central nervous system (CNS) angiitis is a rare inflammatory disorder. To date, clinical data are lacking and treatment remains a matter of debate. The aim of this study is to analyse the main characteristics, response to therapy and outcome of 32 patients with CNS angiitis. Single-centre retrospective study in a tertiary centre was made. Diagnosis of CNS angiitis was made by cerebral angiography and/or magnetic resonance angiography and/or CNS biopsy. The main features and outcomes of primary and secondary CNS angiitis were compared and predictive factors of a favourable outcome were searched. Thirty-one patients (median age 45 Q1-Q3 37-54) sex ratio F/M 2.1) were included. Main clinical features were hemiparesis (35.5 %) and headache (29 %). The median CSF protein level was 0.64(0.52-0.81) g/L and was superior to 1 g/L in six cases. CNS magnetic resonance (MR) imaging findings were most frequently ischemic (96.8 %), bilateral (83.9 %), multiple (87.1 %) and supratentorial (96.8 %). The MR angiography was abnormal in all cases. Among the 31 patients in the study, 19 (61.3 %) were diagnosed with primary CNS angiitis. Systemic lupus erythematosus (n = 6) and vasculitis (n = 4) were the most frequent aetiologies of secondary CNS angiitis. No difference was evidenced between primary and secondary CNS angiitis. Steroids were administered in 79.2 % of treated patients and combined with immunosuppressants in 79.2 % of cases. Eight cases of CNS angiitis relapse were noted. CNS angiitis remains a severe illness. Treatment often associated steroids and immunosuppressants, and diagnosis delay is significantly associated with a poorer prognosis.