PURPOSE OF REVIEW: The purpose of this review is to update the role of the orthopedic surgeon in the management of Down syndrome as these patients are living longer and participating in sporting activities. RECENT FINDINGS: Approximately 20% of all patients with Down syndrome experience orthopedic problems. Upper cervical spine instability has the most potential for morbidity and, consequently, requires close monitoring. Other conditions such as scoliosis, hip instability, patellar instability and foot problems can cause disability if left untreated. In some of these conditions, early diagnosis can prevent severe disability. SUMMARY: Surgical intervention in children with Down syndrome has a high risk of complications, particularly infection and wound healing problems. Careful anesthetic airway management is needed because of the associated risk of cervical spine instability.