A 13-year-old, 6.25-kg (13.8-lb) female mongrel was referred to the authors’ hospital with an acute-onset left pleurothotonus and intermittent opisthotonus. Neurological examination showed clinical signs consistent with a multifocal brain lesion, and neoplastic, vascular, inflammatory and infectious conditions were considered. Precontrast and postcontrast CT images of the brain were obtained and revealed a precontrast hyperattenuating lesion with ring enhancement affecting the left frontoparietal junction. There was severe perilesional oedema, moderate midline shift, and left caudal transtentorial and foramen magnum herniations. A neoplastic lesion with a recent haemorrhagic component was therefore suspected. Following medical treatment with a mannitol bolus and prednisone for eight days, the neurological examination was considered normal. A dark-grey capsulated mass was removed during a left frontoparietal craniectomy. Immediate postsurgical CT confirmed complete excision. Biopsy results were consistent with a cerebral vascular hamartoma. At 27-month follow-up, the dog was clinically normal.