Background Nasopharyngeal carcinoma (NPC) is a frequent head and neck cancer in southern China and Southeast Asia.The majority of NPC patients are managed by radiation oncologists, medical oncologists and head & neck surgeons.Actually, neurosurgeons also play a critical role under specific circumstances.In this article, we described our experience as neurosurgeons in the management of NPC patients.Methods Medical records of NPC patients who received neurosurgical procedure at Sun Yat-sen University Cancer Center were reviewed.Results Twenty-seven patients were identified.Among 27 cases, neurosurgical procedures were performed in 18 with radiation-induced temporal necrosis, 2 with radiation-induced sarcoma, 4 with synchronous NPC with primary brain tumors, 2 with recurrent NPC involving skull base,and 1 with metachronous skull eosinophilic granuloma, respectively.Initial misdiagnosis were found in 6 (22.2%) patients.Conclusion: For NPC patients with intracranial or skull lesions, the initial diagnosis can be occasionally challenging.Special attention should be paid because unawareness of these entities can result in misdiagnosis and subsequent improper treatment.We emphasized the indispensible role of neurosurgeons to facilitate diagnosis and treatment in the multidisciplinary team managing NPC patients.