A case is presented of a 16-year-old anticoagulated, immunocompromised male presenting with a blind, proptotic left eye, total ophthalmoplegia, and central retinal artery occlusion. Ophthalmic examination revealed other features of the orbital apex syndrome. Mucormycosis misdiagnosed as a traumatic orbital hemorrhage led to the patient's death. In this review, differential diagnosis of the orbital apex syndrome (loss of cranial nerves II, III, IV, ophthalmic division of V, and VI) is outlined, and features, diagnosis, and treatment of mucormycosis are discussed. Although mucormycosis is most commonly seen in diabetics, it should be considered in any immunocompromised patient presenting with the constellation of features of orbital apex syndrome.