Rhinocerebral mucormycosis is the most common fungal infection and is frequently seen in patients with poorly controlled diabetes mellitus. We report a rare case of cavernous sinus mucormycosis in a 70-year-old man with insulin-dependent diabetes mellitus. An endoscopic surgical removal of the mucocele was performed. The histopathologic examination revealed characteristic aseptate branching hyphae with invasion of blood vessels, which is consistent with zygomycosis. The patient was administered high-dose amphotericin B therapy. While undergoing amphotericin-B treatment, the patient developed a left-sided hemiparesis. Magnetic resonance imaging and magnetic resonance angiography revealed acute infarction in the right hemisphere and occlusion of the right internal carotid artery in the right cavernous sinus. The patient was managed with conventional medical treatment for acute cerebral infarction and additional amphotericin B infusion. The patient survived, but with severe neurologic sequelae despite extensive medical therapy, including complete-visual loss, leftsided hemiparesis, impairment of cognitive function, dysphagia, and neuropathic pain on the left side of the body. We report herein the clinical course and treatment of a patient with cerebral complications due to mucormycosis and also present a brief literature review.