Listeria monocytogenes (L. monocytogenes) is a rare, but important bacterial pathogen causing central nervous system infection in the elderly, pregnant women, and immunocompromised patients. A 60-year-old man with diabetes presented with headache, fever and rapidly progressive ophthalmoplegia. Based on serological and MRI studies, he was diagnosed with rhombencephalitis due to L. monocytogenes. The patient recovered without complications with urgent initiation of empirical antibiotics and the pathogen-specific antibiotic treatment. L. monocytogenes should be considered as a cause of rhombencephalitis presenting as external ophthalmoplegia.