Background Acute invasive fungal rhinosinusitis (AIFRS) is an aggressive, potentially fatal disease that can spread rapidly to the orbit and intracranial structures causing significant mortality and morbidity. Objective In this study, we present a 10-year experience from a tertiary academic medical center of patients presenting with AIFRS. Data on presentation, mortality rate, comorbidities, surgical, and medical management were analyzed. Methods A retrospective chart review was performed in a tertiary academic medical center of patients with AIFRS from January 2009 through February 2019. Data collected included demographics, presenting symptoms, comorbidities, immunosuppression status, endoscopic and imaging findings, orbital and intracranial complications, surgical and medical management, as well as outcomes and mortality. Results A total of 34 patients were identified. In our series, mortality was noted to be 61.8%, excluding patients who were lost to follow-up. The most common presenting symptoms included facial pain, ophthalmologic complaints, headaches, and proptosis. Only 4 of the 34 patients did not undergo surgical intervention, as they were not deemed surgical candidates; they all succumbed to their disease. Twenty-six of the 30 surgical patients (86.7%) underwent endoscopic sinus surgery, 8 underwent an open approach (26.7%), while 7 patients underwent orbital exenteration (23.3%). All patients had surgical pathology consistent with AIFRS. Fungal species isolated from culture included Aspergillus, Mucor/ Rhizopus, Candida, Cunninghamella Scedosporium boydii, Paecilomyces, and Scopulariopsis. Medical therapies included intravenous amphotericin B, caspofungin, posaconazole, voriconazole, isavuconazole, and micafungin. Conclusion AIFRS was associated with 61.8% mortality in our series of 34 patients over the past 10 years. Early diagnosis, as well as rapid and aggressive surgical and medical management, is necessary for optimal outcomes in this devastating disease.