Intramuscular myxoma of the head and neck is a rare lesion. The tumor demonstrates a benign clinical course without a tendency to recur and is occasionally associated with fibrous dysplasia. It thus deserves to be considered in a separate class from other soft tissue myxomas. The etiological, clinical, tomographic, and pathological characteristics of this tumor are reviewed. We also conclude the location, diagnosis, treatment and prognosis of this tumor.