Brain stem encephalitis is an unusual form of CNS listeriosis that is associated with a high mortality. This is a case of a 46 year-old male with a history of dermatomyositis on methotrexate who presented with fever, headache, assymetrical cranial nerve palsy and right hemiparesis. MRI showed a ring-enhancing lesions in medulla oblongata. CSF cultures grew Listeria monocytogenes. Despite treatment with ampicillin and gentamicin, the patient developed three rare manifestations of neurolisteriosis namely rhombencephalitis, hydrocephalus and brainstem hemorrhage and did not survive. Early detection and treatment is vital in preventing adverse outcomes.