An odontogenic myxoma is a rare, benign tumor that is found almost exclusively in the facial bones, usually the mandible. The diagnosis poses a challenge because its features overlap with those of other benign and malignant neoplasms. We present an unusual case of odontogenic myxoma that involved the maxilla, and we review the clinical, radiographic, and histologic characteristics of this case. Even though it is benign, odontogenic myxoma can be locally invasive and cause significant morbidity. Complete surgical excision is the treatment of choice, but it can be challenging because of the tumor's indistinct margins.