This case report describes an unusual diagnosis of central nervous system botryomycosis in a horse. A 16-year-old Welsh Section D gelding was evaluated for acute onset of hypermetric ataxia, leaning to the left and head tilt to the right. Based on the neurological signs, a cerebellar lesion with accompanying vestibular disease was suspected and supportive therapy consisting of antimicrobial and glucocorticosteroid drugs and hypertonic saline was instituted. This resulted in marked clinical improvement over a 48 h period. Computed tomography performed in the standing, sedated horse following initial stabilisation identified extensive sclerosis and lysis of the right temporal and occipital bones, consistent with an infectious or neoplastic process. Based on the grave prognosis for survival despite the clinical improvement, euthanasia was undertaken. Post mortem magnetic resonance imaging identified a mass lesion impinging on the right cerebellar hemisphere, sclerosis of the temporal and occipital bones lateral and ventral to the mass, as well as destruction of the temporal bone between the inner ear and the cerebellum. These changes corresponded to the presence of a mass within the right dorsal temporal bone, extending into the right lateral temporal bone. The mass extended to compress and adhere to the right lateral hemisphere of the cerebellum. A histopathological diagnosis of botryomycosis was made, affecting the temporal and occipital bones and compressing the cerebellum.