Background Cervical spine instability broadly refers to compromise of the articular congruity. It can be stratified according to spinal level, functional compromise, and mechanism of instability. Conventional wisdom advocates for use of bracing and physical therapy with only a subset of patients proceeding to obtain surgical treatment. Objective The purpose of this review article is to summarize the current state of knowledge on upper cervical ligamentous instability. Methods The literature search was performed in Mendeley. Search fields were varied until redundant. All articles were screened by title and abstract and a preliminary decision to include an article was made. The full-text screening was performed on the selected articles. Any question regarding the inclusion of an article was discussed by 3 authors until an agreement was reached. Results Many articles report on the etiological factors including ligamentous laxity, traumatic injury, syndrome instability, iatrogenic instability, congenital, and inflammatory causes. A few recent studies elucidate new findings regarding pathoanatomy through the use of finite element analysis. A few articles demonstrate the diagnosis and show that radiographs alone have a low diagnostic rate and that functional MRI may be able to better quantify instability. Conservative treatment has been described, but there are no outcome studies in the literature. Surgical treatment has been described in many different populations with good radiologic and clinical outcomes. Recently the use of preoperative 3D CT reconstruction has been described with radiographic and immediate postoperative patient-reported outcomes. Conclusion The presentation of upper cervical spinal instability can be asymptomatic, symptoms of isolated instability, symptoms of nerve irritation, vertebrobasilar insufficiency, or severe neurologic compromise. 3D fine element analysis models and motion-capture systems have the potential to increase our understanding of the pathoanatomic cascade in both traumatic and non-traumatic cases of upper cervical spinal instability. A few modalities on the horizon could increase diagnostic potential. More efforts are needed regarding the use of fine element analysis in understanding the pathoanatomic cascade, the long-term outcomes of children over a spectrum of syndromic causes, and the potential of preoperative virtual simulation to improve surgical outcomes.