A 26-year-old right handed female was admitted to hospital with right homonymous hemianopia associated with alexia without agraphia. Her cranial magnetic resonance imaging and magnetic resonance angiography revealed a left occipital venous infarction due to thrombosis of the left transverse, sigmoid sinuses and the left internal jugulary vein. The underlying conditions were protein C and protein S deficiency associated with the use of oral contraceptives. To our knowledge, alexia without agraphia has never been described due to a venous infarction associated with hereditary thrombophilia in the literature.