Background: Nasopharyngeal carcinoma (NPC) is an extremely rare malignant lesion among the pediatric age group. The relative rarity of pediatric NPC makes the diagnosis difficult. This rarity is often associated with delayed diagnosis which may lead to advanced loco-regional disease. Here, we study the clinical presentations, investigations, and treatment of nasopharyngeal carcinoma in the pediatric age group in a non-endemic region. Result: This is a retrospective study where 21 pediatric patients were enrolled with age under 18 years. They were managed at a tertiary care teaching hospital between December 2010 and January 2019. Majority of the patients in this study were boys (66.7%). All children diagnosed with NPC were treated with radiotherapy covering entire nasopharynx and some children with chemotherapy. Until the patient is in a late stage, most children diagnosed with NPC were presenting with symptoms of neck mass (90.5%), bleeding from the nose (66.7%), nasal blockage (57.1%), and hearing loss (47.6%). Pathological report revealed WHO type III in the majority of the patients. All patients were treated with radiotherapy to primary and enlarged neck nodes. Conclusion: Children with NPC have excellent survival except for those with distant metastatic disease. NPC in the pediatric age is usually not suspected clinically until patient in late stage. The TNM staging has the most relevant prognostic factor. Unfortunately, NPC tends to be locally advanced at the time of diagnosis in the pediatric age group and is sometimes associated with distant metastasis. In our study, most children were diagnosed with NPC along with neck node enlargement and were treated with radiotherapy. The diagnosis of pediatric NPC should prompt timely treatment.