Abstract: Hemangioendothelioma is a rare borderline or low-grade malignant angiogenic tumor, which mainly occurs in the soft tissue, liver, lung, bone and other parts of the body. It is generally infiltrative growth and occasionally metastasis. Ocular hemangioendothelioma is a more rare type of hemangioma, and there is no unified diagnosis for its characteristics and lack of treatment guidelines. Ocular hemangioendothelioma is mainly located in the orbit, but sometimes also in the eyelid and lacrimal organ. The clinical manifestations were eyelid swelling and exophthalmos. Imaging examination often showed local infiltration and growth of tumor with destruction of orbital bone, and systemic metastasis was rare. Most of the pathological changes were vascular endothelial cell proliferation. Immunohistochemical staining showed that CD31 and CD34 were positive. The main treatment is local complete resection of the tumor, but the bleeding tendency of the tumor should be paid attention to during the operation. Surgical combined with radiotherapy and chemotherapy is still a safe treatment for primary orbital hemangioendothelioma. (Int Rev Ophthalmol, 2021, 45: 357-363)

Key words: hemangioendothelioma