Invasive fungal rhinosinusitis (IFRS) is a fatal disease of the nose and paranasal sinuses that typically affects immunocompromised patients. Data on this disorder, which is rare and difficult to diagnose, are lacking in the literature. We collected comprehensive data from 9 patients (7 males, 2 females) with a mean age of 34 years (range: 6 to 58) with IFRS who were treated at our center and examined the factors associated with successful treatment. The parameters examined were patient demographics, disease characteristics, clinical course including surgical and medical therapy, treatment, fungal species involved, and long-term survival at follow-up. The mean duration of symptoms was 33 days. The most common presenting symptoms were loss of vision (67%), headache (55%), and nasal discharge (33%). The middle meatus, middle turbinate, and sphenoethmoidal recess were the most commonly involved sites. Imaging criteria were not definitive in diagnosing IFRS. Early aggressive endoscopic debridement of involved structures was done in all patients with supplementary medical treatment with antifungal agents, which led to complete recovery in most patients. In the remaining patients, subsequent debridement was needed along with more aggressive medical treatment with more than one antifungal agent concurrently. Mortality was 0% after follow-up of 1 year. IFRS is rare, difficult to diagnose, and can present with inconsistent symptoms. Aggressive surgical treatment along with medical treatment should be considered in these patients for complete recovery.