An 18-year-old female presented to the emergency department with a 4-day history of refractory fever and right facial pain. Personal history was uneventful, except she had been diagnosed with thyrotoxicosis and given methimazole two months ago. The day after hospitalization, the patient developed black crusting on the lower eyelid associated with diffuse swelling of the periorbital and the right cheek, and she was unable to open her right eye (Figure 1a). A computed tomography scan of the sinuses showed an opacified right maxillary sinus with right orbital involvement (Figure 1b, asterisks). Upon admission to the hospital, laboratory studies showed she had agranulocytosis (leukocyte counts of 0.33*10 9 /L); the methimazole was immediately discontinued, and supportive therapy with granulocyte colony-stimulating factor was started, but the cellulitis progressed. Pathologic examination of the right middle turbinate presented the necrosis and fungal invasion of the sinus mucosa with hematoxylin and eosin (H&E) stain (Figure1c, black arrowheads). Fungal culture (lactophenol blue) showed numbers of multinucleate primary conidia with prominent papillae on top of unbranched conidiophores (Figure 1d). The characteristic of the pathogen is thin-walled, wide, pauci-septate, ribbon-like, and irregularly branched hyphae with Gomori-Grocott stain of the right nasal swab (Figure 1e). The outcome was fatal on the fourth day of hospitalization, despite the fact that the patient was treated with caspofungin and functional endoscopic sinus surgery.Disseminated infections due to Entomophthorales are extremely rare, not to mention related to drug-induced agranulocytosis (Shaikh et al., 2016;Kimura et al., 2011;Seigel and Hodak, 2012). Given Splendore-Hoeppli phenomenon is the result of host immune response, it usually does not exist in patients with low immune function, as in this case (Vilela and Mendoza, 2018). Figure 1a is a challenging picture because it usually occurs in mucormycosis and aspergillosis (Kara et al., 2015). This case report underlines that Entomophthorales may cause a fatal infection. For this kind of patient, it has been pointed out that multidisciplinary team care treatment is needed to achieve good results (Janappriya et al., 2018). More understanding of this mycosis is warranted for correct diagnosis and proper therapeutic treatment. Figure 1. (A) The patient's facial presentation; (B) A computed tomography scan of the sinuses; (C) Pathologic examination of the right middle turbinate; (D) Fungal culture (lactophenol blue) shows; (E) Gomori-Grocott stain.