Rhino-orbital cerebral mucormycosis is a rare but potentially severe fungal infection in children with high rate of morbidity and mortality. In pediatric patients, uncontrolled diabetes mellitus is considered to be a predisposing factor only in 15% of cases. To prevent and reduce mortality rate of this severe disease, early diagnosis based on clinical findings and biopsy is highly recommended. Herein, we report a case of rhino-orbital cerebral mucormycosis in a 12-year-old girl with type 1 diabetes to demonstrate that a multimodal management approach, involving early surgery which consists in frequent endoscopic sinus debridement and appropriate antifungal therapy, is essential to effectively reduce the spread of infection and achieve effective outcome.