Intramuscular myxoma (IM) is a rare mesenchymal tumor of the head and neck region. The current study reports a case of a 45-year-old man who presented with a painless neck mass. Imaging showed involvement of the levator scapulae and scalene muscles. Core needle biopsy was consistent with intramuscular myxoma. Surgical excision was performed and follow-up for 30 months showed no recurrence. The present study includes a systematic review of head and neck IMs, with a summary of the clinical and demographic parameters of all reported cases in the head and neck region. Surgery was curative in 28 of the 29 published cases, as well as in the current case (96.7%), with the lone recurrent tumor cured following re-resection. Females constituted 57% of the cases and the mean age was 49.7±20.4 years. Although uncommon, IM should be considered in the differential diagnosis of deep neck masses, and surgical excision is the treatment of choice with a low risk of recurrence.
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