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The hair-on-end (HOE) sign is a rare finding seen in the diploic space on skull radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) with the appearance of long, thin vertical striations of calcified spicules perpendicular to the bone surface that looks like hair standing on end. It is classically seen in children/adolescents with hemolytic anemias, in particular, thalassemia major and sickle cell disease. Here, we present a 9-year-old Ugandan girl who presented with stroke in whom head CT demonstrated cerebral intraparenchymal hemorrhage and multiple infarcts on the left with HOE sign. Hb electrophoresis confirmed the diagnosis of sickle cell anemia.