Background <p>Unlike ischemic stroke, hemorrhagic stroke in patients living with sickle cell disease is rare; still the associated mortality and morbidity are relatively high in both HIC and LMIC. Early diagnosis and prompt multidisciplinary management are key components to improve survival and mitigate neurologic deficits.</p> Case presentation <p>We presented the case of a 14-year-old boy with sickle cell disease (SCD) who was admitted with signs and symptoms evoking acute neurologic impairment and elevated intracranial pressure without any history of head trauma. Initial assessment with a non-contrast CT scan revealed a large left temporoparietal hematoma and an associated midline shift. After conservative care, subsequent craniotomy and evacuation of hematoma were performed on day 5. The patient gradually recovered postoperatively and was completely exempt from any presenting symptoms by postoperative day 7. After a meticulous literature review, we found only a few case reports and case series on SCD hemorrhagic stroke, and most of them have not reported similar management strategies and outcomes. For this reason, we deemed it necessary to share our experience and findings in order to help narrow the knowledge gap in SCD hemorrhagic stroke management.</p> Conclusion <p>This single case of a rare but serious complication of SCD in the pediatric population underscores the need for accurate screening tools to detect brain vasculature damage early in the SCD population, as transcranial Doppler ultrasound used for ischemic stroke has not shown satisfying results for SCD hemorrhagic stroke. Such tools will help in classifying those patients by risk group in order to permit prophylactic care for patients at high risk of hemorrhagic stroke. Before the implementation of effective screening strategies, early detection and diagnosis, and prompt multidisciplinary management remain fundamental to reducing SCD hemorrhagic stroke mortality and morbidity.</p>