<b>Background:</b> Fahr syndrome is a rare neurological disorder characterized by bilateral intracranial calcifications, particularly involving the basal ganglia. Ocular manifestations are uncommon but may significantly affect visual function.<br> <b>Case presentation:</b> We report the case of a 45-year-old patient presenting with progressive bilateral visual impairment associated with neurological symptoms including memory disturbances and extrapyramidal manifestations. Ophthalmologic examination revealed decreased visual acuity and bilateral posterior subcapsular cataract. Brain computed tomography demonstrated bilateral calcifications involving the caudate and lenticular nuclei. Laboratory investigations revealed severe hypocalcemia, hyperphosphatemia, and decreased parathyroid hormone levels. The patient underwent sequential cataract surgery by phacoemulsification with intraocular lens implantation, resulting in significant improvement in visual acuity.<br> <b>Conclusion:</b> Cataract represents a rare ocular manifestation of Fahr syndrome. Early ophthalmologic evaluation and surgical management can significantly improve visual outcomes and quality of life.