OBJECTIVE: To study the clinical features and treatment outcomes of head and neck rhabdomysarcoma (RMS) in children. METHODS: The clinical data and results of follow-up visits for 39 children with head and neck RMS were retrospectively reviewed. The children (23 males and 16 females) with a median age of 6 years old (ranged 3 months to 14 years) were admitted to the Beijing Tongren Hospital between November, 2004 and November, 2010. RESULTS: The 39 children mainly presented with exophthalmos and eyelid swelling (56%, 22/39), rhinostegnosis and nasal bleeding (28%, 11/39) and check mass (15%, 6/39). Common primary sites were the eyelid and orbit (56%, 22/39), followed by the nasopharynx and ethmoid antrum (28%, 11/39). Thirty-seven of the 39 patients showed a definite pathologic type and the embryo type was the most common (89%, 33/37). Follow-up visits were carried out for 35 children, with a median follow-up time of 38 months (10-80 months). Of the 35 children, 4 cases received surgery alone, 1 case received chemotherapy alone, 12 cases received surgery plus chemotherapy, 2 cases received surgery plus radiochemotherapy, 13 cases received surgery, chemotherapy and radiochemotherapy (8 cases received 125I particles implants), 2 cases received surgery, chemotherapy, radiochemotherapy and autologous peripheral blood stem cells transplantation (APBSCT), and 1 case received chemotherapy and APBSCT. Seven cases relapsed and 5 cases died of brain metastasis. The total survival rate was 86% (30/35), the complete remission rate was 66% (23/35), and the partial remission rate was 20%. In the 8 cases receiving 125I particles implants, 6 survived without tumor. CONCLUSIONS: Exophthalmos and eyelid swelling are the main presentations in children with head and neck RMS. Common primary sites of this disease are the eye and nasopharynx. The most common pathologic type is embryo type. Comprehensive treatment, including chemotherapy, surgery, 125I particles implants and APBSCT therapy, can improve outcome.