眼眶泪腺淋巴上皮癌是一种非常少见的恶性肿瘤。本例为一位46岁女性患者，表现为左眼睑红肿、眼球突出和视力下降，临床和影像学表现类似于泪腺炎型假瘤。手术完整切除后送检病理检查，其特征是未分化的癌巢之间有大量分化成熟的淋巴细胞、浆细胞浸润，确诊为淋巴上皮癌。术后行化学治疗和局部放射治疗，随访6.5年无复发。眼眶泪腺淋巴上皮癌的临床表现容易与泪腺炎型假瘤相混淆，应该进行病理学检查以明确其恶性表型，完整切除后辅助放化学治疗有较好的效果。

Lymphoepithelial carcinoma of the orbital lacrimal gland is a rare carcinoma. A 46-year-old female patient presented with red and swollen left eyelid, proptosis and blurred vision. The clinical and imaging manifestations were similar to inflammatory pseudotumor. Pathological examination was performed after complete resection.Pathological features showed undifferentiated carcinoma nests surrounded by obvious infiltration of non-neoplastic lymphocytes and plasma cells, which was consistent with the diagnosis of lymphoepithelial carcinoma.Chemotherapy and local radiotherapy were performed after the resection. The case did not relapse after 5 years of follow-up. The clinical manifestations of orbital lacrimal lymphoepithelial carcinoma were easily confused with inflammatory pseudotumor. Pathological examination should be performed to clarify its malignant phenotype.Adjuvant radiotherapy and chemotherapy after complete resection had a good effect.