Multilobular tumour of bone (MTB) is an uncommon tumour and is usually located in the skull. A 13-year-old mixed breed dog was presented with a two-week history of progressively worsening vestibular dysfunction and cognitive abnormalities; it appeared demented and showed asymmetric ataxia and hypermetria of all limbs. The owner opted to have the animal euthanised. Necropsy revealed a large mass occupying the right occipital, parietal and temporal bones, severely compressing the cerebellum and the right occipital lobe. Histologically, it was characterised by the presence of multiple lobules containing osteoid or cartilage and separated by fibrous septae, features typical of MTB. Lung metastases were evident. To our knowledge, this is the first report of an MTB causing both severe cerebral and cerebellar compression and the second detailed report of an MTB of the occipital bone. MTB should be included in the differential diagnosis of bone tumours as well as in cases with central vestibular disease.