Odontogenic myxomas (OMs) are benign mesenchymal locally aggressive neoplasms of the jaw bone Although OMs predominantly involve the mandible, maxillary tumors are usually more aggressive than mandibular tumors. The present study describes the case of a 37-year-old male with a large odontogenic myxoma of the bilateral maxillae, which caused a defect in the right skull base bone. The tumor was successfully removed through radical resection of the hard tissue and local resection around the envelope of the soft tissue. The tumor exhibited no recurrence. However, the current methods for bilateral maxillary reconstruction to restore the maxillary buttress and achieve an optimal aesthetic appearance are complicated due to the lack of suitable conditions for oral rehabilitation with good dentition.