Mucormycosis is a life-threatening disease, were rhinocerebral infection is most commonly seen in the clinical setting. Chronic mucormycosis is a rare presentation that exhibits a challenging diagnosis. We describe the case of a 47 year old diabetic man with complains of left zygomatic arch swelling of 3 months evolution. He had received previous antibiotic treatment without improvement. Biopsy of maxillary sinus revealed the presence of non-septated, 90° angle branched hyphae compatible with zygomicetes. The patient was treated with surgical debridement and amphotericin B until there was no evidence of fungi in the tissue by biopsy. We reviewed chronic rhino-orbito-cerebral mucormycosis from 1964–2014 and 22 cases were found, being this th...