Odontogenic myxomas (OM), also called odontogenic fibromyxomas, are rare tumours of the jawbones. They are benign tumours that can be locally invasive and aggressive, but seldom undergo malignant transformation and do not give rise to metastases. They are often asymptomatic and present as painless swelling. We present herein the case of a 24-year-old patient, with an incidental finding of a maxillary lesion. The lesion expanded to the surrounding soft tissues of the face, the maxillary sinus, the nasal cavity and the ethmoidal cells. After confirmation of OM on biopsy, the patient underwent an extended right radical en bloc maxillectomy with immediate reconstruction of the inferior orbital rim. A literature review of clinical symptoms, radiological presentation, differential diagnosis and surgical management of OM is discussed.
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