Introduction: The nose and paranasal sinuses are known to be affected by a wide variety of inflammatory, neoplastic, and infectious conditions but the symptoms in each are very similar, thus making the clinical diagnosis very dicey. Case description: This is a report of one such rare case of a middle-ages female who presented with recurrent symptoms fitting into several differentials and was difficult to decipher, each presentation leading to a new diagnosis, when we stumbled upon atrophic rhinitis like symptoms, nasal septal perforation, palatal erosion and later to Wegener's granulomatosis or now known as granulomatosis with polyangiitis (GPA) and finally we found it to be a case of NK T-cell lymphoma of extra nodal variety. The challenges faced in the diagnosis and management have been described in detail. Discussion: Wegener's granulomatosis is itself uncommon in India owing to less reporting and less availability of screening tools which has led us to be more ignorant about its presentation and also its differentials. Both GPA and NK T-cell lymphoma resemble each other closely in histology and need a high degree of suspicion and expertise to differentiate, they have very different treatments and prognoses. We must also distinguish them from the more commoner disease of the tropics which is tuberculosis. Conclusion: The extranodal NK T-cell lymphoma also known as a lethal midline granuloma, which was initially thought to be Wegener's granulomatosis due to their resemblance and the challenges faced in arriving at a final diagnosis emphasizes the rarity of such a case and need for all of us in otorhinolaryngology to familiarize ourselves which such cases, to increase clinical suspicion and also knowledge regarding presentation and management to ensure quick lifesaving treatments as this is a lethal disease.