Background: Heterotopic ossification (HO) of the elbow can occur following direct trauma, brain injury, or burns. Development of elbow HO is sporadic, making levels 1-3 clinical evidence difficult to establish. We systematically reviewed literature regarding management and outcomes of surgically treated elbow HO. Methods: A systematic review of the literature regarding elbow HO was performed to compare imaging modalities, surgical timing, surgical approaches, and methods of prophylaxis in outcomes of patients treated with excision. Results: Our systematic review included 24 level 3 or 4 studies investigating 384 post-trauma (158), brain injury (105), or burn (94) patients with elbows complicated by HO that were treated with surgical excision. Average patient age was 36.9 years and there was a 65/35 M/F ratio. For all etiologies, preoperatively elbow flexion/extension averaged 53/83; postoperatively elbow flexion/extension significantly improved to 22/123. Regardless of the etiology, surgical excision of elbow HO significantly improved functional range of motion. Neither total body surface area (TBSA) burned for burn patients or Garland classification for brain-injured patients correlated with outcome. Overall complication rate was 22.6% and included HO recurrence (11.9%), ulnar nerve injury, infection, and delayed wound healing. Conclusion: Surgical treatment of elbow HO leads to improved functional outcome, whether the etiology of bone formation was direct elbow trauma, brain injury, or thermal injury. (C) 2013 Journal of Shoulder and Elbow Surgery Board of Trustees.