Rhinocerebral mucormycosis is a well-described fulminant fungal infection that typically presents acutely in patients with diabetic ketoacidosis or immunosuppression. Chronic presentations of rhinocerebral mucormycosis have also been described. In the chronic infection, the disease course is indolent and slowly progressive, often occurring over weeks to months. The authors report 2 cases of chronic rhinocerebral mucormycosis (CRM) treated at their institution and review 16 other cases reported in the English-language literature. In these cases, the median time from symptom onset to diagnosis was 7 months. The most common presenting features of CRM are ophthalmologic and include ptosis, proptosis, visual loss, and ophthalmoplegia. CRM occurs predominantly in patients with diabetes and ketoacidosis. The incidence of internal carotid artery and cavernous sinus thrombosis is higher in CRM patients than in those with the acute disease, although the overall survival rate for CRM patients is 83%. CRM is clinically distinct from chronic Entomophthorales infection.