OBJECTIVE Mucormycoses are a group of invasive infections caused by filamentous fungi of the Mucoraceae family, with the rhinocerebral form of the disease being the most common in large case series. In the present paper we review the characteristics of the rhinocerebral form of the disease. METHODS The present review is based on the analysis of the current literature on rhinocerebral mucormycosis. BACKGROUND Rhinocerebral mucormycosis is associated with immunocompromised patient state, haemochromatosis, desferrioxamine therapy and prolonged corticosteroid therapy. Uncontrolled diabetes and increased serum iron are regarded as the two leading predisposing factors for the development of the disease. Currently, treatment for the disease is based on three main principles; rapid reversal of underlying predisposing factors, antifungal therapy with amphotericin B and timely surgical intervention. CONCLUSIONS Antifungal drugs of the azole group and new iron chelating agents--deferasirox, deferiprone--have been supported as alternative options to amphotericin B or as salvage therapy. CONCLUSIONS Rhinocerebral mucormycosis requires a high level of awareness if early diagnosis and treatment is to be achieved. Large scale evaluation of arising treatment options is a mandatory course of action in the future research of the disease.