The purpose of this study was to evaluate the role of combined modality treatment in patients with paediatric nasopharynx cancer (NPC). Forty-six patients with paediatric NPC were retrospectively analysed. Forty-four of 46 patients received combined modality treatment. Five-year overall survival and progression-free survivals were 70% and 72% for the whole group, and only three of 46 patients had loco-regional relapse. Complete remission was obtained in 18 of 45 patients (40%), and the overall survival (94% vs. 62% and 19%, p = 0.0009) and disease-free survivals (93% vs. 70% and 16%, p = 0.0002) were significantly better in complete responders when compared with the patients who had partial response or stable disease. The 5-year overall survival and disease-free survivals of the patients who received neoadjuvant chemotherapy (CT) and radiotherapy (RT) followed by CT were superior to the other groups (77% and 80%, respectively). The number of total CT cycles (p = 0.0001), nodal stage (p = 0.05) and treatment response (p = 0.0009) were significant prognostic factors for overall survival. The treatment type (p = 0.02), the number of total CT cycles (p = 0.0006), nodal stage (p = 0.05) and treatment response (p = 0.0002) were found as significant prognostic factors for disease-free survival. The survival of patients receiving six or more CT cycles was also significantly better than that of patients receiving less than six cycles (p = 0.0001). In patients with locally advanced paediatric NPC, CT should be added to RT to improve outcome. However, a standard protocol is yet to be identified, and further studies evaluating the addition of interferon or immunotherapy to CT and RT shall be performed.