Four infants were referred for congenital unilateral corectopia. In each case, the abnormal position of the pupil was caused by a fibrous structure that tethered the iris pupillary margin to the peripheral cornea. No patients showed characteristics of intrauterine infection, Rieger anomaly, ectopia lentis et pupillae, or iris coloboma. Amblyopia was not present in any of the patients. Three children demonstrated progression of the corectopia in the first 6 months of life. Two who developed shallow anterior chambers were treated surgically, one with an Nd:YAG laser and the other with incisional surgery. The third was treated with medical mydriasis. All four children have had favorable visual outcomes to date. The origin of the tethering strands is unclear but may be related to incomplete regression of vessels from the embryologic vascular system. We recommend medical or surgical intervention for unilateral corectopia when the pupillary aperture is displaced peripheral to the central visual axis or when the position of the iris threatens angle structures. Prophylactic occlusion therapy may also be indicated.