1. An unusual congenital anomaly of the cervical spine is described. This lesion caused a localised cervical kyphosis and resulted in the development of a mild tetraparesis.

2. The case reported is believed to be the first on record in the English literature of multiple posterior hemivertebrae in the cervical region.

3. The neck deformity was associated with an unusual combination of developmental anomalies–namely, brachyphalangy and bilateral congenital optic atrophy.

4. The importance of differentiating between congenital and acquired causes of kyphosis is emphasised.

5. The radiographic appearances of posterior hemivertebra are described, and the differential diagnosis is considered.

6. The development of the vertebral body, and the relationship between coronal cleft vertebra and posterior hemivertebra, are discussed. The possible role of a disturbance of vascular supply in pathogenesis is mentioned.

7. This report augments the growing literature on congenital skeletal anomalies occurring in combination with isolated congenital ocular defects.