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Iron deficiency anemia is an unusual etiology of ischemic stroke in the adult population. Only a few case reports exist describing this rare cause of cerebrovascular accident due to low circulating levels of oxygen. To the best of our knowledge, we believe we have described here the first patient presenting with an ischemic stroke secondary to severe anemia without an obvious source of blood loss, evidence of thrombus formation, or associated thrombocytosis. A 28-year-old man presented to our institution, complaining of severe fatigue and was admitted under the pretext of dizziness, blurred vision, esotropia, diffuse retinal hemorrhage, and Roth spots found in the left eye. Laboratory investigations reported a hemoglobin level of 1.12 g/dL (13.5-17.5 g/dl) and thrombocytopenia with a platelet count of 16,000/ µL (150,000-450,000). Concomitant magnetic resonance imaging (MRI) of the brain revealed a right 4 mm periaqueductal lesion. After several red blood cell transfusions, the patient hereafter underwent an improvement in his clinical symptoms. Given no past medical history of anemia or thrombocytopenia, an extensive workup was prompted, but only microcytic anemia likely due to iron deficiency was found. Regarding a stroke in the young adult population, a differential beyond atherosclerosis and hypertension must be considered. Coagulopathies, vasculitis, sickle cell anemia, cerebral venous thrombosis, cocaine abuse, and systemic hypoperfusion are less common, but well-documented, causes of ischemia. Although infrequently mentioned, we demonstrate here a real possibility of severe iron deficiency anemia causing ischemic stroke in the brainstem of a young man. While a thorough neurologic evaluation is not often considered in patients presenting with signs and symptoms of severe anemia, vigilance regarding focal neurologic deficits should prompt suspicion for ischemic stroke in patients with significantly low hemoglobin levels.