Abstract.A 4-year-old male Pekingese dog was referred to the clinic with a history of recurrent seizures and progressive abnormal gait and behavior, which did not respond to treatment. At necropsy, a large cortical defect in the right temporo-parietal cortex, malacia of subcortical white matter, right basal nuclei, and capsula interna, as well as abnormalities of the right hippocampus were observed. Histological examination of the brain revealed moderate to severe nonsuppurative meningoencephalitis in the left cerebral hemisphere and extensive infarction-like lesions with milder inflammation in the right hemisphere. In the right hippocampus, the pyramidal cells were arranged in a gyrus-like pattern and intermingled with gemistocytic and fibrillary astrocytes. The histopathological features of the inflammatory lesions were consistent with necrotizing meningoencephalitis and resembled those described in so-called Pug dog encephalitis. The hippocampal changes were interpreted as dysplasia (monolateral hippocampal cortical hamartia), unrelated to clinical signs and necrotizing inflammatory lesions.Key words: Dysplasia; hamartia; necrotizing meningoencephalitis; Pekingese; Pug dog; encephalitis.The association of noncorrelated dysontogenetic and inflammatory lesions of the central nervous system is an exceptional finding. We describe a necrotizing meningoencephalitis and infarction-like lesions in a Pekingese dog associated with a cortical hamartia of the right hippocampus.A 4-year-old intact male dog was referred to the clinic for small animals at the Faculty of Veterinary Medicine, Pisa University, Italy, with a history of seizures and abnormal gait. Vaccinations, including annual boosters, against distemper virus, parvovirus, leptospirosis, and canine adenoviruses had been given at appropriate time intervals. No major medical problems, with the exception of neurological signs for the last 6 months, had ever occurred. On neurological examination, the most conspicuous findings were a head tilt to the left and a tendency to circle to the left. Proprioceptive positioning responses were delayed in the left limbs and normal in the right. Menace responses were absent on the left and normal on the right. Pupillary light reflexes, as well as