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All the ten patients in our study had the characteristic "dish-" like face and nine patients had acral anomalies. The median Nurick grade improved from preoperative (median = 4) to follow-up (median = 3). The requirement of anti-spasticity drugs decreased in seven patients and the neck-pain improved in nine patients. The median satisfaction at follow-up was good (median PSS = 2); five patients were going back to school. Craniovertebral junction instability in LS is rare and surgically challenging. Early posterior fixation showed a pro