IntroductionThe possible appearance of congenital fusion of the second cervical vertebra with adjacent cervical vertebrae, along with its epidemiology, embryological development, and clinical manifestations, was the aim of the current study.MethodsThe osteological material of 93 dried second cervical vertebrae of both sexes were examined in order to identify the likely presence of congenital fusion with the neighboring vertebrae.ResultsAmong 93 axes, we identified one case of a congenitally fused second cervical vertebrae with the third and fourth cervical vertebrae, which accounted for a frequency of 1.08%. There was an incomplete fusion of the vertebral bodies and almost complete fusion of the laminae and facet joints.ConclusionThe knowledge of such rare vertebral synostosis is crucial for the neurosurgeon, orthopedist, and physician dealing with the cervical spine, as well as the anesthetist when performing procedures, such as endotracheal intubation.