Background: Nasopharyngeal carcinoma (NPC) is a rare malignancy in children and adolescents. The aim of the present study is to evaluate clinical characteristics and treatment outcomes of NPC. Methods: We retrospectively analyzed the data of 8 patients who were treated for NPC at Seoul National University Children’s Hospital between 1998 and 2012. Results: A total of 8 patients (5 males and 3 females) were included with the median age at a diagnosis of 12 (range 9-13) years. The main presenting symptoms were neck mass, nasal obstruction, nasal bleeding, otalgia, hearing defects, tinnitus and headache. One patient had stage II, 2 had stage III, 5 had stage IV diseases. The histologic subtypes were undifferentiated carcinoma (WHO type III) in 4 , squamous cell carcinoma (WHO type I) in 2 and non-keratinizing carcinoma (WHO type II) in 2 patients. All patients were initially treated with cisplatin (100 mg/m2 iv q 4 weeks for 4-6 months), bleomycin (15 unit/m2 iv q 1 week x7) and fluorouracil (1,000 mg/m2 iv q 4 week for 1 year) schedules. Seven patients (87.5%) received radiotherapy with doses of 45-54 Gy at the primary site and neck nodes. Six patients (75.0%) achieved complete remission, 1 (12.5%) partial remission and 1 patient (12.5%) showed disease progression. Five patients (62.5%) developed neurotoxicity related to fluorouracil, and they changed regimen to cisplatin, epirubicin and bleomycin. Four of the 5 patients recovered from neurologic damage after discontinuing fluorouracil. One patient who showed disease progression died of progressive disease without response to chemotherapy and radiotherapy. Treatment-related mortality occurred in 1 patient with septic shock after chemotherapy. Secondary osteosarcoma developed in 1 patient at 6 years after the end of treatment. Overall survival of all patients was 75.0% with a median follow-up duration of 27.5 (range 4.5-287.6) months. Conclusion: Children and adolescents with advanced NPC had a relatively good survival rate through the combination of systemic chemotherapy and radiotherapy.