We present a case of extradural hemorrhage, a rare and unusual complication associated with Sickle Cell Disease, from Nizwa, Oman. A 12-year old male child with Sickle cell disease (SD) was admitted to hospital with backache and pain in both lower Limbs. Clinical examination revealed an average built child with mild pallor, mild tenderness in the lower back and both legs. Rest of the examination was unremarkable including the Central Nervous System. The next day, the patient developed generalized convulsion and became unconscious with shallow breathing. CT of the brain revealed large extradural hemorrhage with shift of mid line structure. After prompt neurosurgical and a hematologic intervention, the child survived and is making progress in rehabilitation. Primary hemorrhagic stroke is a known but uncommon complication of sickle cell disease in childhood, but extradural hemorrhage is an even more rare complication.