BACKGROUND Tumoral calcinosis is rarely located in spine. A 55-year-old Japanese woman with cervical tumoral calcinosis is presented, along with a review of the literature relating to tumoral calcinosis in the spine. We discussed the etiology, diagnosis, and management of this condition. CASE PRESENTATION We report a case of a patient with cervical tumoral calcinosis with end-stage renal disease. A computed tomography scan showed a lobulated, calcified mass around the right facet joint at the fourth-fifth cervical spine and calcifications were also observed in the right intervertebral foramens at fourth-fifth cervical spine and fifth-sixth cervical spine levels and the anterior wall of the spinal canal. By performing a cervical decompression and stabilization, the patient recovered from her neurological symptoms. CONCLUSIONS Although tumoral calcinosis is rarely located in the spine, it should be considered in the differential diagnosis of spinal lesions. If a calcified mass causes acute neurological symptoms, resection of the mass is still the most important treatment.