A 36-year-old woman with an uneventful medical history was admitted to the emergency department following an initial generalized seizure. Neuroimaging workup disclosed a homogeneous mass at the vertex, which first was diagnosed as vertex meningioma. Anticonvulsant drug therapy was administered and the patient was discharged. Two months later the patient was examined in our neurosurgery department for additional therapeutic recommendations. A repeated neuroimaging examination showed considerable regression of the lesion. The findings on magnetic resonance imaging were consistent with those of a regressing extradural hematoma (EDH). A complete blood-coagulation study displayed no evidence of abnormality. Thorough questioning of the patient revealed no history of pericranial infection or head trauma occurring within the last 2 years. The final diagnosis was spontaneously occurring vertex EDH. In this report the authors describe the clinical and neuroimaging features of the case as well as the management strategy, and discuss etiological aspects within the context of a careful review of the literature.