The case of a 65-year-old woman with polyneuropathy, organomegaly, skin changes and monoclonal gammopathy of IgG-lambda type is described. This patient developed an acute carotid obliteration during oral anticoagulation and despite absence of vascular risk factors. Macroangiopathy has been described as a rare systemic manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal spike and skin changes), affecting the coronary and lower limbs arteries. To our knowledge, this is the second case of POEMS syndrome with a cerebrovascular manifestation.