Orbital apex syndrome is characterized by vision loss from optic neuropathy and ophthalmoplegia due to the involvement of ocular motor nerves in the anatomical region of the orbital apex. Patients could present with signs and symptoms deriving from the involvement of structures within the orbital apex, the superior orbital fissure or the cavernous sinus. The primary focus of the ophthalmologist should be to locate the lesion and then identify its etiology. Clinical evaluation holds key to diagnosis which is aided then by certain serological and lab investigations and neuro-imaging modalities including brain and orbital MRI (Magnetic Resonance Imaging) with contrast, CT (Computed Tomography) scans. In rare instances, a biopsy may be needed to aid in diagnosis. Treatment depends on what the nature of the lesion is with inflammatory conditions usually responding to steroids and infections to anti-microbial agents. Through this review, the authors attempt to decode the approach to localizing the lesion, the etiopathology and the management of cases of orbital apex syndrome.