The case histories are reported of two patients with systemic lupus erythematosus (SLE) who developed fatal neurological involvement, each presenting with cerebellar and brainstem signs. Neuro-pathological changes were confined to the cerebellum and brainstem, with clear evidence of a small vessel vasculopathy. The predominant histological abnormalities were multiple perivascular haemorrhages in one case and classical vasculitic changes in the other.