上颈椎肿瘤是指发生在上颈椎部位的原发性肿瘤和转移性肿瘤，临床少见。上颈椎解剖结构复杂，外科治疗难度大、风险高。该文在介绍上颈椎肿瘤流行病学特点、诊断、外科分期的基础上，对该类型肿瘤的治疗原则、手术指征、术式选择、手术入路、微创技术、稳定性重建及内固定等外科治疗进展进行综述。“,”Being primary or metastatic tumors involving upper cervical spine, upper cervical neoplasms are not very common. Surgical treatments for the neoplasms are difficult with high risks because of complex anatomic structures of upper cervical spine. In this paper, epidemiologic features, diagnosis as well as surgical staging for upper cervical tumors were described, and progress in surgical treatments were reviewed, including therapeutic principles, surgical indications, operation choices, approaches, minimally invasive techniques, cervical spine stability reconstruction and internal fixation.