Purpose: To describe a case of a patient presenting with acquired acoria and iris pearls, a rare eye manifestation and pathognomonic finding for leprosy; to reinforce clinical, histopathological, and therapeutic aspects of ocular involvement in leprosy. Methods: Case report. Case description: A 62-year-old male presenting with acquired acoria and iris pearls in both eyes due to leprosy also had anterior uveitis and cataract. Histopathological diagnosis of iris pearls was confirmed by the presence of Hansen’s bacilli. Ophthalmological examination revealed improvement of the visual acuity after iridectomy and extracapsular cataract extraction. Conclusions: To our knowledge, this is the first reported case of acquired acoria in a leprosy patient. It led to impaired vision and reversible blindness. Proper diagnosis and ophthalmological treatment of patients with these conditions are essential for the maintenance of a good quality of life.