The authors report on a 31-year-old man with spontaneous craniocervical osseous fusion secondary to cervical dystonia (CD). After an 8-year history of severe CD, the patient developed a fixed rotation of his head to the right. Three-dimensional computerized tomography reconstructions revealed rotation and fixation of the occiput and C-1 relative to C-2, which was similar to that seen in atlantoaxial rotatory fixation. There was abnormal ossification of the odontoid facet joints and ligaments. Additional ossification was observed in the cervical soft tissue bridging the lateral mass of C-1 and the occiput. The patient underwent partial myectomy of the dystonic left sternocleidomastoid muscle and selective posterior ramisectomy of the right posterior neck muscles; postoperatively he experienced relief of his neck pain. In patients with CD refractory to conservative treatment, the appropriate timing of surgical treatment is important.