Rhino-orbital-cerebral mucormycosis (ROCM) is a rare but devastating fungal infection caused by filamentous fungi of the family Mucoraceae. We report a rare case of unilateral ROCM in a diabetic patient where central retinal artery occlusion (CRAO) was the first manifestation of the disease. Magnetic resonance imaging scan revealed orbital and intracranial spread of the disease. Definitive diagnosis was established by culture of the biopsy specimen which showed Rhizopus oryzae on Sabouraud's dextrose agar. The patient was successfully treated with extensive debridement of sinuses and intravenous liposomal amphotericin B; however, the left eye remained blind following the CRAO.