Central pontine myelinolysis (CPM) is a rare syndrome generally linked to hyponatremia. Autopsy and imaging studies described demyelination in the central pons, in some cases combined with thalamic, internal capsular, hemispheric and cerebellar white matter lesions. The clinical syndrome and prognosis spans from asymptomatic patients displaying only imaging lesions to coma and death. Frequent findings are pyramidal tract and pseudobulbar signs. No effective treatment is known. Alcohol abuse is often associated. The imaging pattern of CPM is unique and generally accepted as diagnostic when other causes such as ischemia, multiple sclerosis, tumors, radiation, or pharmacological effects are ruled out. However, the exact mechanism of this selective process of demyelination remains obscure. The current major controversy concerns the role of therapeutic efforts of sodium level restoration in the etiology of CPM. We present a case of CPM where no hyponatremia was found. We hypothesize that (1) iatrogenic sodium restoration may not in all cases of CPM be the putative mechanism, (2) the clinical onset of CPM may in some patients represent a delayed response to an initially asymptomatic demyelination, and (3) normal admission sodium levels do not exclude the diagnosis of CPM.