Sites in the head and neck region are among the most frequent locations of rhabdomyosarcoma (RMS) in patients younger than 15 years. However, comparable neoplasms in adults are very uncommon. We present a case report of a 27-year-old man who was diagnosed as having RMS. RMS rarely presents in the head and neck of adults, but should be considered in the differential diagnosis of a small cell neoplasm in patients during the third and fourth decades of life.