A one-year-old, female, Maltese dog was presented with head tilting, horizontal nystagmus, and tetraparesis. Blindness was first identified, and magnetic resonance imaging (MRI) scanning revealed diffuse lesion which was hyperintense on T2-weighted image over the cerebellum and brainstem. The immunosuppressive therapy had been administered, but the patient had no improvement. Re-performed MRI revealed the progression of the pre-existed inflammatory lesions. Treatment with prednisolone, leflunomide, cyclosporine, and cytosine arabinoside was initiated. However, neurological signs had been progressive, and the patient was euthanized. The histopathological examination revealed the disseminated granulomatous meningoencephalomyelitis (GME). This GME case suggests the importance of initiation of treatment at the appropriate time.