Abstract: Objective: To investigate the clinicopathological features, diagnosis, prognosis, and clinical significance of epithelioid hemangiomas in the oral and maxillofacial region. Methods: The case history and clinicopathological features of six patients with epithelioid hemangioma in the oral and maxillofacial region were reviewed. All sections were observed microscopically by gross examination and HE staining. The immune structure was analyzed by immunohistochemistry staining and related literatures were reviewed. Results: Six patients were three males and three females, and the average age was 26-year old. The tumors were located around the ear (n=4) and palatine (n=2). All sections were manifested as superficial single mass and one patient with history of trauma. Histopathologically, the lesions were characterized by proliferation of capillaries and small blood vessels, and vascular endothelial cells were present as epithelioid cells or histiocytes. Chronic inflammatory cells could be observed in the whole lesion and most of them could be eosinophilics. For immunohistochemistry, vascular endothelial cells were positive for Vimentin, CD31 and CD34, SMA shows perivascular positive, S-100, Pan CK staining were negative in all the cases. Only one case recurred after surgery and there was no metastasis in all of the cases. Conclusion: Epithelioid hemangioma is a benign vascular lesion, and may be related to local trauma. The effective treatment is the locally complete excision and it is particularly liable to relapse if not completely removed. Accurate preoperative and intraoperative diagnosis is very important for complete surgical resection.