Purpose: We report an unusual case of rapid and severe anterior capsular contraction associated with secondary intraocular lens (IOL) dislocation following cataract surgery in a patient with unspecified rod-cone dystrophy. Case Report: A 68-year-old woman with a history of uncharacterized bilateral rod-cone dystrophy presented with blurry vision 1 month after cataract surgery. Best corrected visual acuity was 20/40 in the operative eye. Slit-lamp exam showed severe anterior capsular phimosis limiting view of the fundus. Our patient underwent 2 sessions of Nd:YAG anterior capsulotomy with limited success. Limited anterior vitrectomy was then performed without success due to densely adherent capsular tissue to the anterior surface of the IOL and additional secondary IOL dislocation. She ultimately underwent pars plana vitrectomy, removal of the capsular bag, and IOL exchange with a scleral fixated IOL. Conclusion: Rapid and severe anterior capsular contraction following cataract surgery is rare but appears to be associated with rod-cone dystrophy.