Objective: To analyze the clinical manifestations of retroperitoneal schwannoma in order to improve the diagnosis and treatment of this rare disease. Methods: Between January 1951 and September 2004, 81 patients with retroperitoneal schwannoma were retrospectively analyzed. Results: All cases received operative therapy. Sixty cases (74.1%) received a total resection; 12 cases (14.9%) subtotal resection, and 9 cases (11.1%) exploration. During the surgical operation, a single tumor was found in 77 cases (95.1%), and multiple tumors in 4 cases (4.9%). Most of the retroperitoneal schwannomas located beside the spine. The tumor was a fusiform, round or oval mass that was sharply circumscribed and encapsulated. Pathologic results showed all 81 cases were benign schwannoma. In the 4 cases of multiple tumors, 2 (2.5%) were diagnosed as double-primary tumors associated with ascending colon adenocarcinoma and lung squamous-cell cancer. One case recurred postoperatively. Conclusion: Retroperitoneal schwannoma was rare and preoperative diagnosis was difficult. Most of retroperitoneal schwannoma were benign and the surgical treatment was the first choice.