Mucormycosis is the third most common invasive fungal infection that particularly occurs in immunocompromised patients. Intracranial and extracranial arteriovenous vasculopathy is a complication that makes this disease more complex and difficult to treat. We describe a 23-year-old female, who presented to her local hospital with acute blindness and diabetic ketoacidosis-induced coma requiring intensive care treatment. She was found to have lesions in the nasal sinuses, orbit, and frontal base. The left carotid artery was occluded from its origin in the neck to the supraclinoid segment and left cavernous sinus involvement. No cerebral infarction was noted. Biopsies obtained by endonasal debridement confirmed mucormycosis. In addition to antimicrobial therapy, she underwent several multidisciplinary approaches to treat her disease. Multiple endonasal, and cranial procedures were done including bilateral orbital exenteration. After prolonged treatment on the intensive care unit she made a remarkable recovery to the point where she was communicating verbally and had normal limb movements and later discharged home. She remained alive and well for two months, but later succumbed to a recurrence of her disease. In conclusion, mucormycosis-induced vasculopathy is a complex problem, which merits aggressive treatment of this invasive disease. It is normally regarded as an indicator of grave prognosis.