Introduction: Sickle cell disease (SCD) is defined as an autosomal recessive disorder characterized by the production of abnormal hemoglobin S and is correlated with high morbidity and mortality. The clinical consequences of SCD include pain crisis, acute chest syndrome, and strokes. Spontaneous epidural hematoma is a rare manifestation in sicklers with few cases reported in the literature.[ 6 ] The pathophysiology is not completely understood. However, a few explanations have been reported over the years that include vaso-occlusion of the bone resulting in bone infarction, microfracture due to rapid expansion of hematopoiesis of the inner cortex, and sludging of the sickle cells in the diploic veins—all result in leaking of blood in the epidural or in the subgalea space. Patient Concerns: A 14-year-old boy known to have SCD (Hb SS) presented to the Security Forces Hospital with a history of diffuse headache associated with nausea that started 12 h prior to presentation. Diagnosis: Computed tomography (CT) showed bilateral frontal epidural hematoma and subgaleal space. Intervention: A multidisciplinary team was created (hematology, neurology, neurosurgery, and interventional radiology) and a plan was formulated as follows: Continuous monitoring of the patient’s neuro vital signs and transfuse the patient with blood and platelets in addition with Levetiracetam. Outcomes: The patient was discharged after 9 days of hospital admission. He has remained symptom-free post-transfusion. Post-discharge CT scan showed a reduction in the hematoma size. Conclusion: A high index of suspicion is needed for a prompt diagnosis and treatment of this rare complication of SCD. The management strategy of EDH depends on the level of consciousness of the patient upon presentation. Surgical approach with craniotomy and evacuation or conservative management have been used with full recovery of the patients.