A 52-year-old patient presented with signs clinically consistent with ectopia lentis et pupillae syndrome. The patient was treated successfully with vitrectomy, dislocated lens removal using perfluorocarbon liquid and phacofragmentation in the vitreous cavity, pupil reconstruction, and scleral-fixated intraocular lens implantation in both eyes. Despite the fact that the surgery was successful in technical terms, the final visual outcome was not as good as expected. This was caused by the optic nerve atrophy resulting from long-lasting glaucoma. Nevertheless, the described surgical techniques may be considered an effective method of treatment in cases of ectopia lentis et pupillae syndrome.