Primary nasal tuberculosis is rare. We report a case that was all the more extraordinary because of the age and sex of the patient (an 11-year-old boy), the unusual associated symptoms (epistaxis and grand and seizures), and the presence of intracranial extension. Clinical and radiologic findings on our initial evaluation suggested that the patient had a large sinonasal malignancy. The patient manifested no evidence of pulmonary tuberculosis. The diagnosis of primary nasal tuberculosis was established only after we obtained the results of histopathology of the excised mass and a subsequent tuberculin skin test; the diagnosis was confirmed by the patient's rapid response to antituberculosis drug therapy. We also review the relevant literature on this rare condition.