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Background: There is a great geographic variation of extranodal natural killer (NK)/T-cell lymphoma, nasal type (NNKTL) prevalence, with a much higher prevalence in the Asian and South American populations. According to our knowledge and searches, only one other case report/study of NNKTL has been published in South Africa. Case presentation: We present a southern-African 31-year-old male residing in a township in the south of Johannesburg, South Africa. He presented with signs and symptoms similar to those of benign upper airway diseases. Further work-up of persisting signs and symptoms yielded a diagnosis of NNKTL. He was treated with the SMILE (steroids — dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide) chemotherapy regimen, and sandwich radiotherapy was planned. He had a partial response to chemotherapy but unfortunately demised due to overwhelming sepsis prior to radiation therapy. Conclusion: Making a diagnosis of NNKTL in resource-limited settings is challenging as the diagnosis requires not only the demonstration of NK-cell markers but also to have a positive Epstein-Barr virus (EBV) in situ hybridization (EBER-ISH). Collaboration of smaller centres with larger and better equipped centres is required to diagnose and document NNKTL more accurately in the African setting.