Although the importance of bilateral cortical innervation in the control of swallowing is well known, neurogenic dysphagia caused by unilateral hemispheric ischemic lesion has been also reported. Our patient is a 41-year-old male who developed difficulty swallowing liquids, oral apraxia, and motor dysphasia, followed by right-hand ataxia. Brain magnetic resonance imaging (MRI) showed subcortical tumefactive conglomerate of cystic lesions in the left frontal, precentral region, which were sharp edged, with perilesional edema, concordant with lesions in Marburg's variant of multiple sclerosis. Steroid treatment and plasma exchange therapy led to disappearance of neurological symptoms. Treatment with interferon β-1a 40 mcg sc was initiated. During a 3-year follow-up, clinical/brain MRI scan showed no new neurological manifestations, a significant regression of lesion size, and no new brain lesions. To the best of our knowledge, this is a first case of dysphagia caused by unilateral hemispheric lesion in a multiple sclerosis patient.