Odontogenic myxoma is a rare benign neoplasm that originates from odontogenic ectomesenchyme. There is no standard of care and recurrences are frequent after conservative surgical procedures. A retrospective study conducted at a single cancer center, with analysis of medical records of all patients diagnosed with odontogenic myxoma from 1980 to 2010, along with a literature review. There were 14 patients with diagnosis of odontogenic myxoma (OM). Most patients were female (78.6%) and Caucasian (100%), with ages ranging from 7 to 51 years (21.6 ± 11.6 years). The time period between the first symptom and first consultation ranged from 0 to 60 months (19.4 ± 19.97 months). The most frequent complaints were increased local volume or failure to tooth eruption. The most common tumor site was the mandible (11 cases, 78.5%). About radiological findings, most lesions were multilocular (9 cases, 64.3%) and with imprecise limits (12 cases, 85.7%). Surgery was performed in all cases and curettage was the most applied technique (10 cases, 71.4%). Three patients underwent mandibulectomy and complex reconstructions including iliac crest microvascular flap. Three patients had postoperative complications and 4 had local recurrences of the tumor. The follow up time ranged from 12 to 216 months (112 ± 70.8 months). All patients are without clinical and radiographic evidence of disease. OM is a locally aggressive and rare tumor. There is no gold standard surgical management and the therapeutic decision should be individualized taking into account the characteristics and extension of the tumor.