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Sickle cell anemia is an autosomal recessive disorder resulting from insertion of the valine codon at position sixth of beta-hemoglobin gene instead of glutamate leading to the production of insoluble beta-hemoglobin chain. Although sickle anemia is mostly associated with bone pain and anemia, other manifestations also occur. Acute head syndrome is an uncommon complication resulting from skull bone infarction, cortical osteopenia, and rupture of tortuous periosteal blood vessels leading to extravasation of blood into the subgaleal space. This report therefore intends to create awareness that nontraumatic scalp swelling is an uncommon complication in sickle cell disease and no traditional practice or surgical excision should be employed in managing it.