Visual anosognosia or Anton-Babinski syndrome is a rare neurological condition related to cortical blindness. The patients deny their blindness and affirm adamantly that they are capable of seeing. The clinical presentation includes confabulations and sometimes confusional states. In this article we report two patients with anosognosia related to ischemic stroke in two different sets of etiology and pathogenesis. We describe the major clinical manifestations of this syndrome and review the current medical literature. Two patients were identified, a 96-year-old male with visual anosognosia secondary to a right posterior cerebral artery thrombosis, and a 56-year-old female with the same syndrome but related to central nervous system angiitis in relation with multiple sclerosis and Hashimoto's thyroiditis. Visual anosognosia or Anton-Babinski syndrome is a rare neurological condition. However the ischemic vascular cerebral disease is a frequent etiology. We believe that this is the first report of this syndrome in relation to angiitis with a clear autoimmune pathogenesis.