Eyelid epithelioid hemangioendothelioma (EHE) is uncommon. The present study reports the clinical, histopathological and immunohistochemical characteristics of a 10-year-old girl with EHE and reviews the related literature. The patient was admitted with a painless red mass on the left eyelid, which had been present for 6 months. Under the microscope, tumor cells from the surgical specimen were composed of epithelioid cells (70%) and spindle cells (30%). The cytoplasm was eosinophilic, and some areas of the cytoplasm showed signet ring-like or vacuolization changes. The presence of red blood cells could be seen in the luminal part of the primary blood vessel. Immunohistochemical results showed that the tumor cells were positive for CD34 and CD31, and the Ki-67 proliferation index was 3%. As the first resection margin was positive, recurrence occurred 3 months after surgery, Therefore, patients with such tumors should undergo full resections to ensure a negative margin to prevent recurrence.