PURPOSE: To evaluate the results of pars plana lensectomy in patients with hereditary lens subluxation. METHOD: Hospital records of patients with hereditary lens subluxation who had undergone pars plana lensectomy at Labbafinejad Medical Center, Tehran-Iran from 1996 to 2003 were reviewed. Patients with more than 6 months of follow up were included. Underlying disorders, best corrected visual acuity (BCVA) before and after surgery, intraocular pressure (IOP), postoperative refraction and complications were evaluated. RESULTS: Overall, records of 87 eyes of 49 patients including 27 male and 22 female subjects were reviewed. Mean follow up duration was 20±18 months. Underlying disorders leading to lens subluxation included Marfan syndrome (79.5%), Weill-Marchesani syndrome (8.2%), simple ectopia lentis (8.2%), and homocystinuria (4.1%). The most common indication for surgery was non-correctable refractive error (92.1%). Mean BCVA was 1.13 LogMAR (20/250) preoperatively, which improved to 0.26 LogMAR (20/30-20/40) postoperatively (P < 0.001). BCVA better than 20/40 was achieved in 82.8% of cases after surgery. Angle-supported anterior chamber intraocular lens (ACIOL) was implanted in 85.1% of the eyes. Prophylactic band was applied in 63 eyes (72.4%). Retinal detachment developed in four eyes (4.6%) and was successfully treated. CONCLUSIONS: Lensectomy/anterior vitrectomy with implantation of an angle-supported ACIOL in patients with hereditary lens subluxation improves vision significantly without considerable complications.