IntroductionBurkitt’s lymphoma is a highly aggressive, small, non-cleaved B-cell non-Hodgkin's lymphoma. In the sporadic form of the disease that occurs in non-endemic areas around the world, most commonly in developed countries, patients usually present with an abdominal mass that frequently involves the ileocecal region of the bowel; ocular or orbital involvement is rare. Primary disease of the sinuses is uncommon and, to the best of our knowledge, that of the anterior septum has never been described. We report the diagnosis and successful management of Burkitt’s lymphoma originating from the nasal septum in a male patient.Case presentationAn otherwise healthy 78-year-old Caucasian man who did not smoke cigarettes was admitted to our Ear, Nose and Throat outpatient clinic with the complaint of nasal obstruction due to left-sided nasal septal thickening. Paranasal computerized tomography revealed a well-circumscribed solid mass originating from his anterior nasal septum and obstructing his airway. The final diagnosis of Burkitt’s lymphoma was verified by immunohistochemical studies. Our patient had a good clinical outcome after chemoradiotherapy, with no problems reported to date in the second year of follow-up.ConclusionWe provide what we believe to be the first report of a case of sporadic Burkitt’s lymphoma involving the nasal septum, and describe the efficacy of first-line chemotherapy. Being an original case report with broader clinical impact across more than one area of medicine, this case presentation has the potential to significantly advance our understanding of Burkitt’s lymphoma and we emphasize the need to include this disease in the differential diagnosis of patients presenting with a nasal septal mass.