In February 2013, two Korean native goats with circling, dysphagia, sialorrhea, and death were submitted for necropsy to the Jeju Self-Governing Provincial Veterinary Research Institute. Histopathologically, multifocal to confluent micro-abscess composed of neutrophils and macrophages and severe multifocal perivascular cuffing were observed in pons, medulla oblongata, and cerebellum. Isolated bacteria from the brain sample were Gram-positive coccobacilli and were confirmed as Listeria (L.) monocytogenes by VITEK and PCR analysis. Based on clinical sign, histopathology and bacterial isolation, this case was diagnosed as encephalitic listeriosis. In our best knowledge, this is the first report of encephalitic listeriosis caused by L. monocytogenes in Korean native goats in Jeju.