Polycythemia vera represents a neoplastic proliferative disorder characterized by an abnormal overproduction of cells of myeloid lineage, especially erythrocytes. It can infrequently present as an acute ischemic stroke as the initial manifestation. This presentation is rarer in young patients
hence, it signifies the essence of a complete evaluation to elicit an accurate diagnosis to help prevent morbidity and mortality. In this report, we present a young male with acute onset hemiparesis who, on imaging, was found to have an ischemic infarct in the posterior limb of the internal capsule. On further investigation, the patient had a markedly elevated hematocrit and hemoglobin, and a mutation in JAK2 on genetic analysis. After confirming the diagnosis, phlebotomy was performed, which brought hemoglobin levels closer to the reference range and provided symptomatic relief.