We report three cases of anomalies of the craniovertebral junction with myelopathy. Case 1 was a 28-year-old man who complained of muscle weakness of his right upper limb and gait disturbance. He was diagnosed as having basilar impression, atlantoaxial dislocation and hyperplasia of the dens with spondyloepiphyseal dysplasia. Case 2 was a 15-year-old woman who complained of gait disturbance. She was diagnosed as having ossiculum terminal and atlanto-axial dislocation with Down's Syndrome. Case 3 was a 67-year-old man who complained of limb numbness and gait disturbance. He was diagnosed as having atlanto-occipital fusion, basilar impression and atlanto-axial dislocation with spongilosis. All patients had surgical treatment with improvement in their cord symptoms.