Torticollis is a clinical sign of either CNS or musculoskeletal dysfunction. Thorough clinical and neuroradiographic evaluation is indicated for determination of the source, course, and exact nature of the deformity before a considered approach to surgical management is undertaken. Torticollis of neurogenic origin is managed by surgical decompression of the brainstem or cervical spinal cord when indicated, as in the presence of hydrocephalus, the Arnold-Chiari malformation, syringobulbia , syringomyelia, colloid cyst of the third ventricle, or neoplasms in the third ventricle, posterior fossa, or cervicomedullary junction. In progressive and medically refractory cases of spasmodic torticollis, neuroablative procedures, neuroaugmentive procedures, selective muscle excisions, and radical cervical muscle excisions have been used to control the dystonia. Torticollis of musculoskeletal origin is often secondary to or associated with trauma, although congenital craniocervical anomalies may be a predisposing factor. The primary goals of surgery for craniovertebral or upper cervical spine deformity are reduction of the malalignment; decompression of the brainstem, cervical spinal cord, and/or nerve roots if necessary; and stabilization of the upper cervical spine.