Tumors that require mandibulectomy in childhood are sarcomas and a group of primary osseous and odontogenic tumors. Among the last group, odontogenic myxoma is a benign tumor, which affects primarily the mandible, locally aggressive and frequently described as a lesion composed by scarce cells star-shaped embedded in a myxoid stroma and the origin of it is still a controversy. Its frequency is 0, 5% of all bone tumors and only 3 to 6% of odontogenic tumors. Common in pediatric patients and adolescents, myxomas are considered slow-growing tumors with infiltrative potential that produces expansion of cortical bone with extensive destruction. Despite its benign nature, this tumor has a high rate of recurrency after curettage. Resection with safety margins is the surgical treatment of choice. Many techniques have been described for the reconstruction of mandibular defects in childhood, within these, the vascularized fibula graft allows the preservation of cosmetics and function. Our case is a 3 year old boy with an odontogenic myxoma which compromised the entire right mandible. He was treated with a complete hemimandibulectomy and an immediate VFFG. To 6 months follow up, the patient shows no signs of recurrence, normal mandibular dynamics, and no sign or symptoms of TMJ dysfunction.

Conflict of interest: Main author is in the ICOMS scientific commission.