Rhabdomyosarcoma in the head and neck is an uncommon tumor found mainly in children. The usual sites of occurrence in the head and neck are the orbit and the parameningeal sites, which include the middle ear, nasopharynx, paranasal sinuses and the nose. The embryonal type is the most common rhabdomyosarcoma of the head and neck region and is usually found in children. The pleomorphic type is rarely seen and usually found in the adult. The overall prognosis is poor, but due to the recent addition of multimodal chemotherapy to the treatment of this tumor, the 5 year survival rate has improved from less than 20% to around 70%. We present a case of pleomorphic rhabdomyosarcoma of the nasal cavity in an adult patient treated by combination of surgery, postoperative radiotherapy and chemotherapy. The clinical and histological findings and recent trends in the treatment of this tumor are presented and discussed.