BACKGROUNDInvasive fungal rhinosinusitis (IFR) caused by Cunninghamella is very rare but has an extremely high fatality rate. There have been only seven cases of IFR caused by Cunninghamella reported in English and, of these, only three patients survived. In this article, we present another case of IFR caused by Cunninghamella, in which the patient was initially treated successfully but then deteriorated due to a relapse of leukemia 2 mo later.CASE SUMMARYA 50-year-old woman presented with a 2-mo history of right ocular proptosis, blurred vision, rhinorrhea and nasal obstruction. Nasal endoscopic examination showed that the middle turbinate had become necrotic and fragile. Endoscopic sinus surgery and enucleation of the right orbital contents were performed successively. Additionally, the patient was treated with amphotericin B both systematically and topically. Secretion cultivation of the right eye canthus showed infection with Cunninghamella, while postoperative pathology also revealed fungal infection. The patient’s condition gradually stabilized after surgery. However, the patient underwent chemotherapy again due to a relapse of leukemia 2 mo later. Unfortunately, her leukocyte count decreased dramatically, leading to a fatal lung infection and hemoptysis.CONCLUSIONAggressive surgical debridements, followed by antifungal drug treatment both systematically and topically, are the most important fundamental treatments for IFR.