1. One hundred cases of congenital elevation of the scapula are reviewed; thirty-four were treated by operation.

2. A method of grading of appearance is suggested to assist in selecting cases for operation.

3. In very mild cases operation does not confer worthwhile benefit.

4. Very severely affected patients cannot be made to look normal.

5. The appearance often deteriorates during a period of rapid growth of the neck, making continued observation and reassessment essential.

6. The primary aim of treatment is to improve appearance; improvement in function is uncertain.

7. Excision of the supero-medial part of the scapula, and of the omo-vertebral bone when present, is recommended in most cases requiring operation. It is safe and simple, with little risk of loss of function and with simple after-treatment.