Uremic tumoral calcinosis (UTC) is a form of metastatic tissue calcification unique to dialysis patients, manifesting with amorphous and cystic masses containing calcium phosphate deposits in periarticular soft tissue. An involvement of the cervical spine with bone destruction is extremely rare in UTC. We describe a 44-year-old uremic female on long-term continuous ambulatory peritoneal dialysis who developed UTC in the peri-odontoid region with consequent atlantoaxial subluxation and spinal cord compression, featuring severe neck soreness, headache, and hypertension. Surgical removal of the destructive cervical spine lesion, showing typical tumoral calcinosis on histology, completely resolved the clinical symptoms. To date, the patient maintains uneventful postoperative course with tight control of serum phosphorus, calcium, and secondary hyperparathyroidism by medical treatment. We also review other reported unusual cases of UTC involving the cervical spine and discuss the differential diagnosis of destructive spinal lesions in uremic patients, such as UTC, dialysis-related amyloidosis, and brown tumors.