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Ischemic stroke is one of the manifestations of reversible cerebral vasoconstriction syndrome (RCVS). Many precipitants and associated disorders of RCVS have been suggested. However, few case reports have indicated an association between anemia and RCVS. Here, we report a case of a 66-year-old female with severe iron deficiency anemia (IDA), who presented with ischemic stroke and cerebral vasoconstriction, which gradually improved with conservative treatment. High-resolution vessel wall magnetic resonance imaging findings and reversibility suggested the possibility of RCVS. In patients with RCVS and ischemic stroke, IDA should be considered. Prompt management should be delivered to prevent disease progression and recurrence.