Abstract: 【Abstract】〓Primary central nervous system lymphoma is a clinically rare non-Hodgkin lymphoma with lymph node external invasion, which only has an involvement of brain, spinal cord and their accessory structures. Because this disease is characterized by low specificity in clinical imaging performance, difficult diagnosis and high misdiagnosis rate, the author reported a case of primary cranial-orbital lymphoma. The clinical presentation, diagnostic procedure, treatment, and outcome of this case were presented with a review of the related literature, aiming at providing references and recommendations for the clinical treatment of similar diseases.

Key words: Neurosurgery, Misdiagnosis, Central nervous system lymphoma, Cranio-orbitaltumors