Objective: The aim of this report was to present a rare case of apparently unilateral Peters anomaly and describe the clinical characteristics, surgical approach, and visual prognosis. Methods: We presented the case of a 7-year-old female patient with posterior corneal defect due to kerato-lenticular adhesions along with anterior dislocation and opacification of the lens in the left eye and a history of post-traumatic evisceration of the right eye. Systemic associations included mental underdevelopment, left torticollis and scoliosis. No family history of acquired or inherited diseases were determined. We performed cataract extraction in the left eye and opted for aphakia. Results: Based on clinical findings, we considered unilateral Peters anomaly type II. Cataract surgery slightly improved the visual acuity from hand moving to 20/ 400 UCVA (uncorrected visual acuity) and 20/ 100 with +10.0 diopters at 1 month postoperative. No enlargement of the corneal opacity was observed. Conclusions: In this case, we were able to diagnose Peters anomaly only in one eye. The diagnosis required long follow-up with periodic measurement of intraocular pressure (IOP) to early detect glaucoma. The complexity and uniqueness of the case relied on the difficult approach made by the cloudy cornea and anterior lens dislocation. We applied a combination of techniques including adhesiolysis, cataract extraction and anterior vitrectomy. Further interventions such as secondary IOL (intraocular lens) implantation or PKP (penetrating keratoplasty) will be taken into consideration after six-month and one-year postoperative follow-up. Abbreviations: PA = Peters anomaly, DM = Descemet's membrane, IOL = intraocular lens, VA = visual acuity, OVDs = ophthalmic viscosurgical devices, IOP = intraocular pression, PKP = penetrating keratoplasty, BCVA = best corrected visual acuity, UCVA = uncorrected visual acuity.