Malignant tumours with nasopharyngeal primary location are relatively rare in children. One of the most frequent paediatric neoplasms in this region is the undifferentiated nasopharyngeal carcinoma tumour (UCNT). These tumours are usually revealed by nasal obstruction, headache or cervical nodal involvement. The nasopharyngeal mass can be discovered during an ear-nose and throat examination and confirmed after medical imaging investigation. Diagnostic is obtained by nasopharyngeal mass or cervical nodal biopsy. This tumor has a high chemo and radio-sensibility in children. Irradiation of the cavum and the nodes areas remains as the reference treatment of this disease. Most paediatric protocols add chemotherapy to radiotherapy. The value of concomitant or adjuvant chemotherapy is still debated in paediatric population. It was shown that the T and N staging at diagnosis determined by the classification TNM is a major prognostic factor. Due to the importance of the loco-regional sequelae after irradiation and the satisfactory global prognosis, the actual trend is a decrease in the total dose of irradiation delivered, particularly for patient with a good response to first chemotherapy. This article sheds light on the particularities of UCNT in paediatric patients.