Peripheral odontogenic myxoma of the oral cavity is extremely rare. We present a case of peripheral myxoma arising from the mandibular anterior lingual gingiva in a 55-year-old man. Intraoral examination revealed a solitary, elastic-hard mass on the lingual gingiva of the mandibular in the range of right first incisor to canine, measuring 13mm in diameter. The mucosa of the mass was partially white in color, and the shape of the mass was slightly irregular. Abnormal uptake FDG on PET/CT (SUVmax 9.9) was observed in the anterior mandible. The mass was excised with a margin of healthy gingiva. The histopathologic diagnosis was peripheral odontogenic myxoma. Follow-up for 2 years after excision has shown no evidence of recurrence.