Marchiafava-Bignami disease (MBD) is a neurological disorder that has been found to be associated with chronic alcoholism and malnutrition. We report a 45 year old man, chronic alcoholic that developed discouragement for activities involving daily living, changes in retrograde memory in addition to mutism and gait instability. Brain MRI showed central atrophy of the corpus callosum(CC), hypointensity(necrosis) and ventricular dilation(white matter and subcortical region involvement). Pathological characteristics include isolated demyelination and axonal loss in the central layer of the CC sparing the dorsal and ventral layer. This pattern of necrosis of the middle layer of the CCis a typical finding in the disease. The clinical diagnosis has considerably changed during recent decades after brain MRI provided the opportunity of a reliable in-vivo diagnosis. With early detection and treatment, the prognosis of MBD may be good. Marchiafava-Bignami disease (MBD) is a rare complication related to alcohol abuse and chronic malnutrition which results in demyelination and symmetrical necrosis of the central layer of the corpus callosum [1]. Serum anion gap and osmotic gap can be observed in patients ingesting etanol. The serum anion gap still might be high, which could lead to the demyelination syndrome. The increase in serum osmolarity produced by alcohol is relied onserum concentration, molecular weights, and metabolic rate. Toxic effects of alcohol and its metabolites can cause demyelination of the corpus callosum [2]. Despite alcohol is the principal element, there are other causes that can be factors such as cyanide and CO poisoning, sepsis, sickle cell disease and Plasmodium infection [3]. The clinical presentation is variable, including mental confusion, dementia, psychosis, spasticity and dysarthria [4]. Since the symptoms are not specific, the clinical diagnosis can be difficult, being the magnetic resonance imaging (MRI) of pivotal value in the investigation [5]. Diffuse thickening of the CC andT1 and T2 prolonged areas in the CC are considered characteristic MRI findings at the acute phase. It has been reported that the first change on MRI is diffuse swelling of the corpus callosum, followed by a genu lesion, and finally by a splenium lesion, sparing the rostrum [5,6]. T1-weighted MRI in the subacute to chronic stage shows hypointense cystic-necrotic lesions in the corpus callosum in addition to callosal atrophy. Extracallosal lesions have been reported involving predominantly the periventricular white matter or the basal ganglia [7].