Objective To summarize the preliminary effectiveness of surgical treatment of Erdheim-Chester disease (ECD), so as to improve the understanding of the disease by orthopedic surgeons. Methods The clinical data of 9 patients with ECD between December 2012 and October 2017 were retrospectively analysed. There were 6 males and 3 females with an median age of 42 years (range, 8-61 years). The disease duration was 4-59 months (mean, 39 months). There were 2 cases of multiple lesions, including 1 case involving soft tissue of the buttocks and bilateral tibia, 1 case involving the sinus, skull base, and proximal right tibia; 7 cases with single lesion, including 3 cases of right femoral neck, 1 case of proximal right tibia, 1 case of right humerus, and 2 cases of ribs. Nine patients were diagnosed according to clinical manifestations, imaging examination, and pathological diagnosis. Four patients underwent needle biopsy before operation and 5 patients were diagnosed by postoperative pathology examination. Five cases underwent lesional scraping and internal fixation, 1 case underwent bone scraping and bone grafting, and 3 cases underwent lesion resection. One of the multiple lesions was treated with interferon and hormone. Results Nine patients underwent the surgery safely. There was no fever, wound exudation, infection, etc., and the incisions healed by first intention. All the patients were followed up 4-59 months with an average of 31.4 months. One patient with bilateral tibia and hip soft tissue involvement continued to receive medical treatment, and the tumor was controlled without significant increasing. The remaining 8 patients were examined for X-ray films at 3, 6, and 12 months after surgery, the bone has been fused and the steel plate and intramedullary nail were firmly fixed, and no tumor recurrence was observed. At 1 year after surgery, the pain symptoms of the patients improved and returned to normal life; 3 of them who involving the right femoral neck walked freely, and the quality of life improved significantly. Conclusion ECD patients can achieve the purpose of eliminating lesions and relieving pain after surgical treatment, and the surgical treatment has the advantages of quick relief of pain, improved quality of life, small side effects, and low economic cost when compared with medical treatment.