目的:综述眼眶NK/T细胞淋巴瘤的临床诊断、治疗及常见误诊原因。方法:结合文献和2例个案,对眼眶NK/T细胞的临床诊断、治疗及鉴别诊断进行分析。结果:眼眶NK/T细胞淋巴瘤是临床罕见的一类结外淋巴瘤,易误诊为眼结膜炎、眼非特异性炎症、眼眶周围软组织炎或蜂窝织炎。病理改变、免疫表型符合鼻型NK/T细胞淋巴瘤。结论:眼眶NK/T细胞淋巴瘤临床表现、免疫表型、影像学特征独特,容易误诊,临床进展快,死亡率高。 Objective: To summarize the clinical diagnosis and treatment of orbital NK / T cell lymphoma and the causes of common misdiagnosis. Methods: Combined with literature and two cases, the clinical diagnosis, treatment and differential diagnosis of orbital NK / T cells were analyzed. Results: Orbital NK / T cell lymphoma is a rare clinical type of extranodal lymphoma, easily misdiagnosed as conjunctivitis, non-specific inflammation of the eye, orbital soft tissue inflammation or cellulitis. Pathological changes, immunophenotype consistent with nasal NK / T cell lymphoma. CONCLUSION: The clinical manifestations, immunophenotypes and imaging features of orbital NK / T cell lymphoma are unique, easily misdiagnosed, rapid clinical progress and high mortality.