Mucormycosis is an infrequent infection caused by opportunistic fungi belonging to the Mucorales order; Mucoraceae family, whose characteristics are vascular invasion by hyphae, which determine thrombosis and tissue infarction. In generally affects patients with underlying diseases and produces serious invasive and often fulminant infections. Some of the risk factors leading to mucormycosis are diabetic ketoacidosis, immunosuppressive therapy, leukemia and lymphomas with prolonged neutropenia. Various clinical presentations are described, relating to anatomical site involved, being the rhinocerebral site, the most frequent one, specially in diabetic patients. Although this infection has a high morbidity and mortality, its prognosis has improved the last years, relating to therapeutic measures like correction of predisposing conditions, use of amphotericin B and early aggressive surgery. It is very important to suspect this infection in patients with predisposing conditions so an early diagnosis can be made.