Background: Chronic calcified subdural empyema is an exceedingly rare central nervous system infection with a handful of cases published to date. Reported cases presented with nonspecific clinical signs and symptoms. The duration between the initial onset of symptoms and diagnosis can vary drastically from a few years to up to 46 years. Although there are known predisposing causes, the initial source of infection can sometimes be difficult to identify. Case presentation: Our patient was a 39-year-old Ethiopian man who presented with left-side body weakness of 6 years' duration with worsening of symptoms of 6 months' duration. He had no history of trauma, meningitis, or previous surgery. The results of routine laboratory tests were normal. The diagnosis was made by computed tomography and magnetic resonance imaging and was confirmed by surgery. Frontoparietal craniotomy was performed, and evacuation of non-foul-smelling collection was done. The patient was reoperated for tension pneumocephalus 48 hours after the initial surgery. He died 10 days later. Conclusion: This is a rare case of a giant chronic calcified subdural empyema with no known preceding history of trauma or infection.
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