Abstract. An 11-week-old, male, Staffordshire Bull Terrier had a history of generalized ataxia and falling since birth. The neurologic findings suggested a localization in the cerebellum. Magnetic resonance imaging of the brain was performed. In all sequences the area of the cerebellum was almost replaced by fluid isointense to cerebrospinal fluid. A complete necropsy was performed after euthanasia. Histologically, the lesion was characterized by extensive loss of cerebellar tissue in both hemispheres and vermis. Toward the surface of the cerebellar defect, the cavity was confined by ruptured and folded membranes consisting of a layer of glial fibrillary acidic (GFAP)-positive glial cells covered multifocally by epithelial cells. Some of these cells bore apical cilia and were cytokeratin and GFAP negative, supporting their ependymal origin. The histopathologic features of our case are consistent with the diagnosis of an ependymal cyst. Its glial and ependymal nature as demonstrated by histopathologic and immunohistochemical examination differs from arachnoid cysts, which have also been reported in dogs. The origin of these cysts remains controversial, but it has been suggested that they develop during embryogenesis subsequent to sequestration of developing neuroectoderm. We speculate that the cyst could have been the result of a pre-or perinatal, possibly traumatic, insult because hemorrhage, and tissue destruction had occurred. To our knowledge, this is the first description of an ependymal cyst in the veterinary literature.Key words: Cerebellum; ependymal cyst; histopathology; MRI findings; traumatic.An 11-week-old male, Staffordshire Bull Terrier with a history of intermittent generalized ataxia since birth was presented for examination. The dog was alert and clinically normal except for a gait characterized by generalized hypermetria and head bobbing with a tendency to fall. There was an obvious intention tremor and decreased proprioception in all limbs without lateralization. Cranial nerve function and spinal reflexes were normal. The neurologic findings suggested a lesion localized in the cerebellum. Magnetic resonance imaging (MRI) of the brain was performed with a 0.3 Tesla MRI unit (Hitachi Airis II; Hitachi Medical Systems, Dü sseldorf, Germany). Sequences included a FSE T2 in sagittal and transverse orientation, a FE 3D T1 in transverse plane, a FE 3D MPR (high-resolution T1w gradient echo), a FLAIR (cerebrospinal fluid-suppression), and a BASG-sequence in dorsal plane. In all sequences the area where the cerebellum is normally located was almost completely filled with fluid isointense to cerebrospinal fluid (CSF) (Figs. 1, 2). The left part of the vermis and the left ventral hemisphere of the cerebellum were reduced to approximately one fifth of their normal volume. These cerebellar remnants were deformed and irregularly shaped with concave and convex surfaces. The fourth ventricle seemed to be separated from the fluid-filled cavity by a thin membrane and appeared normal in size. There were no signs ...