Synovial chondromatosis is an uncommon disorder characterized by the formation of multiple cartilaginous nodules within the synovium, most commonly affecting large joints. Its involvement with the spine is rare; only six cases have been reported. The authors describe two patients with synovial chondromatosis involving the cervical spine. In the first case, synovial chondromatosis arose from the left C1-2 facet joint. This patient underwent a two-stage procedure including a posterior approach for tumor resection and occipitocervical fusion as well as a transmandibular circumglossal approach to the anterior craniocervical junction to complete the tumor removal. Interestingly, on histopathological examination, scattered foci of low-grade chondrosarcoma were intermixed within the synovial chondromatosis. To the authors' knowledge, this is the first report of secondary low-grade chondrosarcoma arising in vertebral synovial chondromatosis. In the second case, synovial chondromatosis involved the left C4-5 facet joint. Tumor resection and cervical fusion were performed via a posterior approach. In this report, the authors describe the clinical presentation, radiographic findings, operative details, histopathological features, and clinicoradiological follow-up data obtained in these two patients and review the literature pertaining to this rare entity.