We report the case of a 53-year-old woman diagnosed with corticobasal syndrome (CBS) due to antiphospholipid syndrome (APS) secondary to systemic lupus erythematosus. Brain MRI showed marked cortical atrophy, several small infarctions in the deep white matter, and mild white matter changes, all of which were probably due to thrombosis manifestations of APS and could also be related to the CBS. To the best of our knowledge, this is the fourth reported case of CBS due to APS. It is noteworthy that although the common underlying pathologies of the CBS are neurodegenerative diseases, either primary or secondary APS can manifest itself as the CBS.