Epithelioid hemangioendothelioma (EHE) is mainly seen in adults: it can be located in superficial and deep soft tissues as well as in substantial organs, such as lung, liver, and bone, but rarely in the head, and its incidence is less than 1% of all intracranial vascular tumors. In this paper, we report a case of primary EHE originating from the cranial lamina cribrosa. EHE is a rare vascular endothelial tumor characterized by epithelioid cells: it occurs in soft tissues and substantial organs, but is rare in the skull. it was first described and named as epithelioid vascular endothelial cell tumor by Weiss and Enzinger in 1982, and is considered to be an intermediate vascular tumor. In the WHO (2007) classification of central nervous system tumors, EHE is classified as a mesenchymal tumor with a junctional or indeterminate biological behavior. It has been reported that the occurrence of this disease is related to chromosome 11q and 12q variants. The clinical manifestations are mainly tumor erosion, destruction of the skull, subscalp and dura mater, rarely growing subdurally, and most of the lesions are prone to hemorrhage, which makes preoperative qualitative diagnosis difficult. Most of the primary EHEs have a good prognosis, but there are 2 cases of postoperative metastasis and 4 cases of death, which indicate that their biological behavior has a certain malignant tendency.