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Epithelioid hemangioendothelioma (EHE) is a rare, locally aggressive well-differentiated endothelial tumor, which frequently develops from the peripheral veins. Meningeal EHE has only been reported in four previous cases. Here we report the case of a 38-year-old female with EHE of the meninges and secondary involvement of the skull. The patient presented with occasional headaches and slightly blurred vision. Computed tomography and magnetic resonance imaging showed a mass under the occipital bone and parietal bone, with involvement of the skull. Total resection of the tumor and involved occipital bone was performed. Histopathologically, the tumor cells had abundant eosinophilic cytoplasm and round nuclei arranged in sheets or in a nested pattern. Multiple vascular spaces containing red blood cells were observed. Intracytoplasmic vacuolization and occasional intraluminal erythrocytes were common. Immunohistochemically, the tumor cells were diffuse, intense staining for vascular markers and patchy immunoreactions for epithelial markers. Local recurrence was observed 1 month after surgery, but no regional or distant metastasis was found until 25 months. To our knowledge, this is the first case of EHE of meninges with skull involvement occurring in a female.