Pathological destruction of the axis vertebra leads to a highly unstable condition in an upper cervical spine. As surgical resection and anatomical reconstruction of the second cervical vertebrae represents a life threatening procedure, less radical approaches are preferred and only few cases of C2 prosthesis are described in literature. The focus of this case report is a 21-year-old man with a pathological fracture of C2 managed primarily surgically with the C1-C3 dorsal fusion. Due to the progression of giant cell tumor and destruction of the axis vertebra, C2 prosthesis through anterior approach and dorsal occipito-cervical fusion C0-C4 were performed. Postoperative infection was managed surgically with a 2-staged dorsal debridement, ostheosynthesis material change and autologous bone graft. After a 4 week-intravenous therapy with the ceftriaxone in combination with the amoxicillin/clavulanate, followed by 12 week per oral therapy with amoxicillin/clavulanate in combination with ciprofloxacin, the complete recovery of the infection was achieved. Radiotherapy was initiated 2 months after the last revision surgery and the patient showed a good clinical outcome with stable construct at a 1 year follow-up. A review of literature of all reported C2 prosthesis cases was performed CONCLUSION: C2 prosthesis allows a more radical resection in pathological processes involving the axis vertebra. Combined with the posterior fusion, immediate stability is achieved. Anterior surgical approach is through a highly unsterile oral environment which presents a high-risk of postoperative infection.