Rheumatoid arthritis (RA) is the most common inflammatory disease of the cervical spine (CS). After hands and feet, CS is the most commonly involved segment, being present in more than half of the patients with RA. Especially in the CS, RA may cause degeneration of ligaments, leading to laxity, instability and subluxation of the vertebral bodies. This is often asymptomatic or symptoms are erroneously attributed to peripheral manifestations. Otherwise, this may cause compression of spinal cord (SC) and medulla oblongata leading to severe neurologic deficits and even sudden death. Owing to its potentially debilitating and life-threatening sequelae, inevitable progression once neurologic deficits occur and the poor medical condition of afflicted patients, CS involvement remains a priority in the diagnosis and its treatment will remain a challenge. The surgical approach aims a solid fixation of the upper cervical spine, giving stability, preventing neurologic deterioration and injury to the SC, leading to improved neurologic function, vascular integrity and maintenance of sagittal balance. The recent advances in surgical techniques, complete understanding of the anatomy and precise preoperative evaluation led to safer and more effective procedures that have decreased complication rates. Based on the fact that when a patient becomes myelopathic the rate of long-term mortality increases and the chance of neurologic recovery decreases, many authors agree that early surgical intervention, before the onset of neurologic deficits, gives a more satisfactory outcome. However, the timing when a prophylactic stabilization should occur is poorly defined, and so, patients with radiographic instability but without evidence of neurologic deficit are still the most difficult to manage.