Odontogenic myxoma (OM) is a relatively rare, benign odontogenic tumor with locally aggressive behavior, but it is a nonmetastasizing neoplasm of the jaw bones. Although radical resection with an appropriate surgical margin is recommended, emerging evidence has suggested that a more conservative approach will result in acceptable recurrence rates with less morbidity if careful long-term follow-up is provided. A 56-year-old Japanese man with odontogenic myxoma of the left mandible was conservatively treated by surgical enucleation and curettage because he desired functional and cosmetic preservation. During a follow-up period of 100 months, the patient has remained clinically and radiologically free of recurrence. As far as we can ascertain, 20 reports published after 1990 described 37 patients with mandibular OM that had been treated by conservative surgery. Tumors recurred during a mean follow-up of 49.2 ± 42.8 months in 7 (18.9%) patients, and only one of five patients who were followed up for over 100 months developed recurrence. The rate of recurrence decreased from 24.0% to 8.3% when follow-up exceeded 60 months. Although enucleation and curettage have proven effective, the risk of recurrence remains considerable and long-term follow-up is indispensable. More evidence of long-term outcomes after conservative surgery for OM is needed.