Purpose: High refractive errors and optical aberrations reduce vision when the lens edge bisects the pupil. We studied outcomes of eyes with ectopia lentis following lensectomy. Methods: Charts of 11 consecutive patients with bilateral ectopia lentis who underwent lensectomy-anterior vitrectomy in at least one eye from 1985 to 2004 were reviewed. Eighteen eyes were operated. One eye was excluded due to short-term follow-up (<2 years). Results: Mean age at surgery was 7.7 years (2 to 17 years). Median follow-up after lensectomy was 10 years (range 2 to 16 years). Six eyes were followed for 6 to 10 years, and another six eyes were followed for 11 to 16 years. Patient diagnoses included Marfan syndrome (nine eyes), ectopia lentis et pupillae (three eyes), simple ectopia lentis (two eyes), homocystinuria (two eyes), and sporadic spherophakia (one eye). Preoperative best-corrected visual acuity (BCVA) ranged from 20/60 to light perception, and postoperative BCVA ranged from 20/20 to 20/100 (14 eyes were at least 20/30). Complications included posterior vitreous detachment (two eyes, 12%), glaucoma (one eye, 6%), transient ocular hypertension (one eye, 6%), wound dehiscence with iris incarceration (one eye, 6%), transient vitreous hemorrhage (one eye, 6%), and peripheral anterior synechiae (one eye, 6%). No retina detached. Conclusions: Our cohort of patients with long-term follow-up shows that pars plana lensectomy can be successful in restoring vision when conservative measures fail.