A 18-month-old, male Maltese dog with acute seizure was referred to Veterinary Medical Teaching Hospital, Kyungpook National University. Abnormal neurologic findings included a tendency to fall to the left, turn and circle to the right, negative bilateral menace responses and pupillary light reflexes, and diminished responses to facial sensation testing on both sides. Radiographic signs were normal, except for occipital dysplasia. Magnetic resonance imaging of the brain revealed bilateral asymmetric ventriculomegaly, which is more on left side. Cerebral edema was also suspected. The dog died after 9 days from the onset of seizure, and a necropsy was performed. Histopathologically, the brain lesions in the meninges and the cerebral cortex of this dog was presented. There was severe diffuse nonsuppurative inflammation in perivascular area of the meninges and the cerebral white matter. Based on the results, this case was diagnosed as necrotizing meningoencephalitis in Maltese dog.