A 39-year-old man with acquired torticollis suffering from cervicobrachialgia and neurological deficits is presented. Due to a change in head position a transient reproducible tetraplegia and severe vegetative dysfunctions were caused. The origin of this uncommon serious combination of symptoms and signs was a chordoma of the upper cervical spine. After surgical decompression the patient was free of neurological deficit and pain. Review of the literature did not reveal any similar case.