Purpose: To introduce the importance and world prevalence of ocular leishmaniasis (OL), its clinical manifestations, and diagnostic and management features with more emphasis on Iranian cases.

Methods: We reviewed all available articles published about OL during recent 50 years.

Results: OL is an ocular protozoal infection which is widespread in the world but is more common in developing countries. All forms of leishmaniasis (cutaneous, mucocutaneous, and visceral) can involve the eye, but ocular lesions are usually seen in cutaneous form. Clinical manifestations of OL include: lid skin ulcer, conjunctivitis, episcleritis, cataract, glaucoma, keratitis, uveitis, and finally eye destruction. Clinical diagnosis of this disease is difficult and any delay in diagnosis and management can cause irreversible damage to the eye and adnexae. From 1950 to 2005 there were limited reports of OL in the literature from: USA , Brazil , Germany , Spain , Turkey , India , Sudan , and Iran . In Iran , four cases were reported, two of whom ended in blindness, because the treatment procedures were not effective. Treatment with combined stibogluconate and allupurinol in early stages of the disease usually leads to complete healing of the lesions and disappearing of parasites from the ocular samples.

Conclusion: Although OL is a relatively rare disease in the world, it is potentially dangerous and affected patients must be followed up closely, especially immunodeficient ones. Early diagnosis of OL and rigorous treatment may prevent blindness. Pentavalent antimonial compounds (combined stibogluconate with allopurinol) can be effective in treatment.