We report a case of secondary chondrosarcoma arising in the ilium showing spontaneous regression after frequent local tumor recurrences followed by multiple surgeries of marginal or intralesional excision. The patient was a 16-year-old boy who had been diagnosed as having multiple exostosis from 9 years of age. He experienced an increasing abdominal tumor mass that formed a huge tumor. Although marginal resection of the tumor was attempted, intraperitoneal dissemination was caused by rupture of the tumor capsule and the peritoneum, as a result of severe tumor adhesion to the peritoneum. During the 5 years after the initial operation, local recurrences occurred seven times in various areas of the intra- or retro-peritoneum and marginal or intralesional excision was performed every time for a total of 14 tumors. However, since the seventh operation, the patient has had no evidence of recurrence or metastasis of the disease for more than 10 years. Therefore, we considered that the cancer might have spontaneously regressed.