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Background: Spontaneous epidural hematoma (EDH) is a rare entity with only few cases reported in literature. Spontaneous EDH can be due to dural vascular malformations, coagulopathies, sinus infections, middle ear or orbital infections and tumours. Although spontaneous EDH as a complication of sickle cell disease is even much rarer. Case report: Author reported a case of bifrontal spontaneous EDH in a 9 year old child who presented with severe headache and vomiting with GCS 12/15 (E3V4M5). NCCT head was done which was suggestive of bifrontal EDH with mass effect. Immediate craniotomy was done and epidural hematoma was evacuated. Patient made a successful recovery. He was later investigated and found to be suffering from sickle cell disease. Conclusion: This case report highlights that a previously asymptomatic sickle cell disease patient can present with spontaneous large epidural hematoma which requires immediate surgical intervention. So, this can be a sole presentation in sickle cell disease in previously asymptomatic patients. Sickle cell disease should always be kept in differential diagnosis in spontaneous extradural haematomas.