Mucormycosis is an aggressive opportunistic infection caused by fungi within the order Mucorales, which includes the genera Rhizopus, Mucor, and Absidia. Most of the reported cases involve immunocompromised patients and different clinical pictures have been observed. The most common form is the rhino-cerebral infection that manifests in the nasal mucosa and in the paranasal sinuses and may involve the ethmoid through the orbital vessels. Despite aggressive medical and surgical treatment, mortality in patients with mucormycosis can be as high as 65%. Here we report a clinical case of a patient with an acute leukaemia successfully treated with a combination of surgery and antifungal therapy.