Rhino-orbital-cerebral mucormycosis (ROCM) is an uncommon but fatal fungal infection. We report a rare case of unilateral ROCM with ipsilateral central retinal artery occlusion and contralateral choroiditis, which later progressed to endogenous fungal endophthalmitis. The patient was successfully treated with sinuses debridement, systemic liposomal amphotericin B, and intravitreal amphotericin B. The endophthalmitis completely resolved with good vision, but the ROCM eye remained blind due to central retinal artery occlusion.