S.L., an 8-year-old boy, presented to us with difficulty walking and using his hands for the past 2 months. He was falling frequently, and his handwriting had deteriorated to a point where he was finding it difficult to hold a pencil. An MRI revealed high cervical spinal cord compression due to congenital basilar invagination. He underwent surgery with a transoral release and posterior occipitocervical fusion. We were able to achieve complete anatomic correction of the deformity. The patient wore a cervical collar for 3 months and went on to make a complete recovery within one year.