We report a case of an 18-year-old male with sickle cell disease presented with vaso-occlusive crisis. About 24 hours after hospital admission for increasing severity of headache, the patient had sudden deterioration of his level of conciseness. An emergency CT scan of the head revealed a large right-sided parietal region extradural hematoma with mass effect and midline shift. The patient underwent emergency surgery and hematoma evacuation, and he made good recovery. The possible pathogenesis of this rare condition is discussed. Trauma to the skull causing injury to the middle meningeal artery, middle meningeal vein, or dural venous sinuses is responsible for most cases of epidural Haematoma (EDH). Spontaneous EDH is a rare entity in clinical practice. Common causes include sinusitis, coagulation abnormalities, dural metastasis, and Langerhans cell histiocytosis. Non traumatic spontaneous epidural Haematoma is a rare and often unmentioned complication of sickle cell disease (SCD). It is often associated with skull bone infarction.

Milankumar Senjaliya has completed his M.Ch. Neurosurgery degree from BJMC Medical College and Civil hospital, Ahmedabad- One of the largest teaching institutes of India. He is presently working as Assistant Professor of Neurosurgery at PDU Medical College, Rajkot, India. He has various international and national paper presentations and publications in field of neurosurgery.