Non-traumatic isolated nasal septal aspergillosis in the absence of sinusitis is a rare but serious infection in immunocompromised patients. Adequate management requires early diagnosis, prompt empiric antifungal therapy, and surgical debridement to prevent progression to life-threatening complications. With the increasing population of immunocompromised children, it is essential for timely management that clinicians have a high index of suspicion for this unusual presentation of aspergillosis.