Odontogenic myxoma can present with variable clinical and radiological appearance; therefore, it should be considered in the differential diagnosis of radiolucent and mixed radiolucent–radiopaque lesions of both jaws in all age groups. Whether it can be classified as a myxofibroma or strictly myxoma depends on the amount of collagen it contains, as myxomas generally present as a mucoid ground substance with little collagen. We report a case of myxofibroma of the maxilla, a rare, though benign but locally aggressive odontogenic tumor. Our patient presented as an asthenic, chronically ill-looking, pale, dehydrated but acyanosed young woman. She presented on account of a six-month history of rapidly progressive large mass of the maxilla with extensive destruction of the maxillary bone and displacement of the associated teeth. There was associated history of spontaneous bleeding and intermittent pain. The mass was initially biopsied and diagnosed as desmoplastic fibroma. Chest radiographic findings revealed thin-walled cavitary lesions with characteristic air-crescent signs which was suggestive of left-sided pulmonary mycetoma. The maxillary lesion was treated by surgical excision with peripheral ostectomy. The literature review was done using search engine PubMed, Google Scholar, and Scopus with the following keywords: “Odontogenic myxofibroma,” “odontogenic fibromyxoma,” “odontogenic myxoma” “myxofibroma of the maxilla,” “myxofibroma of the mandible,” “myxofibroma of the jaw,” “fibromyxoma of the jaw” “case report on myxofabroma” and “case series on myxofibroma.” Articles where the amount of fibrous and myxoid tissue were not quantified were excluded from the study.
