Mucormycosis is the term for infections caused by fungi of the order Mucorales. These fungi may produce a severe disease in susceptible individuals, notably immunocompromised patients with diabetes, acidosis and receiving desferrioxamine. Rhinocerebral mucormycosis is a clinical form which is caused by these fungi. If this form of the disease is not treated, it can rapidly progress and cause death. Diabetic ketoacidosis is more important than hyperglycemia alone in rhinocerebral mucormycosis. If the other diseases which cause immunodeficiency are also present, the prognosis of the disease is worse. This case report describes a patient who is diagnosed as rhinocerebral mucormycosis associated with non acidotic diabetes and myelodysplastic syndrome. This case also was administered desferrioxamine to decrease the elevated serum iron level. The di-sease rapidly progressed and patient died although aggressive therapy (surgical and medical) was performed.