Myxoma is a benign tumor of mesenchymal origin. It frequently occurs in the muscles of the hip and extremities; however, it rarely occurs in the head and neck region. This report describes the second case of an intramuscular myxoma of the tongue. A 23-year-old woman was referred to our institution for the diagnosis and treatment of a left tongue lesion. T2-weighted magnetic resonance imaging revealed an 8 × 6-mm mass in the tongue. Based on a clinical diagnosis of a tongue tumor, excisional biopsy was performed under general anesthesia. The histopathological diagnosis was an intramuscular myxoma. The postoperative course was uneventful, and there was no evidence of tumor recurrence 3 years after surgery.