Objective Pediatric invasive fungal rhinosinusitis (IFS) is a devastating infection that manifests almost exclusively in immunocompromised children. The goal of this work was to determine which clinical features carry prognostic value for survival. Methods A retrospective review of children with a histopathological diagnosis of IFS was performed at an academic tertiary care institution from 1990 to 2021. Clinical variables were collected to generate survival and life‐table estimators at 6‐months and 1‐year. Results Eighteen patients were included in this analysis, with a mean age of 9.8 years (range, 1–17 years). Most children were neutropenic (n = 15, 83.3%), with acute lymphoblastic leukemia (n = 10, 55.6%) representing the most common primary diagnosis. A mean of 3.2 operations (range 1–7 operations) was performed per patient for either mucormycosis (n = 10, 55.6%) or aspergillosis (n = 8, 44.4%). The mean time to absolute neutrophil count recovery was 65.8 days (range 20–137 days), with a 6‐month and 1‐year survival rate of 47.6% and 41.7%, respectively. Gross total resection (p = 0.006, p < 0.001), number of antifungals (p = 0.0004, p = 0.0003), and total operation number (p = 0.0032, p = 0.0035), served as positive prognostic factors for 6‐month and 1‐year survival. Conversely, altered mental status (p = 0.0026), cerebral involvement (p = 0.0010), cranial neuropathies (p < 0.0001), hyperglycemia (p = 0.0445, p = 0.0208), and intensive care unit status (p = 0.0013) served as negative prognostic factors for 6‐month and 1‐year survival. Conclusion Several key elements were identified and found to play a vital role in influencing survival for pediatric IFS. Early diagnosis, prompt medical therapy, and aggressive surgical intervention remain at the forefront in the treatment of this complex opportunistic infection. Level of Evidence 4 Laryngoscope, 133:1239–1250, 2023