Aim: Burkitt's lymphoma (BL) is an aggressive type of B-cell non-Hodgkin's lymphoma. BL is common in paediatric age in regions where malaria is holoendemic. Its incidence is also high in immunosuppressed patients in non-endemic regions, especially when associated with HIV infection. Primary BL at the nasopharynx in the paediatric age group is extremely rare, especially in non-endemic areas. It often results in misdiagnosis and delayed treatment. BL becomes fatal if untreated or treated late. The aim of this study is to evaluate the clinical presentations, investigations, and treatment of nasopharyngeal BL among paediatric patients in a non-endemic region. Material and methods: This was a retrospective study in which 21 paediatric patients with nasopharyngeal BL were enrolled, with age under 18 years. This study was done between July 2010 and August 2020. Results: There were 21 children with nasopharyngeal BL included in this study; 17 male and 4 female, with a male-to-female ratio of 4.25:1. The most common presenting symptoms were nasal block (100%), epistaxis (71.42%), rhinorrhoea (66.66%), snoring (57.14%), and headache (42.85%). The most common location of the nasopharynx was the roof of the nasopharynx (28.57%), lateral wall (23.805), posterior wall (19.04%), and junction of the roof and posterior wall (28.57%) of the nasopharynx. All the children were treated with methotrexate, vincristine, cyclophosphamide, doxorubicin, and cytarabine. The mortality of the children with BL in this study population was related to longer duration of clinical manifestations and also misdiagnosis before getting the final diagnosis of BL. Conclusions: Paediatric patients with primary nasopharyngeal BL have an excellent survival rate. Early diagnosis and prompt treatment improve the prognosis of nasopharyngeal BL.