Cervical spondylotic myelopathy usually arises in patients in their late 40s or early 50s, most frequently at the C5/6 and C6/7 levels. Recently, excellent results have been attained with microsurgery in cases of cervical spondylosis. On the other hand, treatment of cervical spondylotic myelopathy in patients with athetoid dystonic cerebral palsy entails several problems. The authors report three cases of such troublesome myelopathy. A 34-year-old male with severe athetoid movement showed cervical spondylotic myelopathy. Myelography and magnetic resonance (MR) imaging demonstrated compression of the spinal cord through the C3-C5 levels. A 47-year-old female with athetoid dystonic cerebral palsy presented myelopathy. Myelography and MR imaging showed instability and spinal cord compression at the C5/6 level. A 34-year-old male with spasmodic torticollis showed C6 radiculopathy due to cervical disc hernia at the C5/6 level. Cervical anterior decompression with interbody fusion brought temporary improvement in all the three patients. However, such problems as slippage of Halo-vest, difficulty in eating during Halo-vest fixation, relapse of neurological deficit, were experienced. Due to postoperative cervical instability, cervical laminectomy is considered to be contraindicated in such patients. Anterior decompression with bone fusion has been reported effective, but, if athetoid dystonia continues, there is a potential for myelopathic deterioration due to spondylotic changes adjacent to the fused vertebrae.