The largest known series of patients with ectopia lentis et pupillae (ELeP) was clinically evaluated. Sixteen patients from eight families were characterized with respect to ocular anomalies. Variability between eyes of the same patient and among different patients was marked. In addition to the well-known but inconstant displacement of the lens and pupil, other characteristics of this protean syndrome included severe axial myopia (and associated fundus abnormalities), poor vision, retinal detachment (RD), enlarged corneal diameters, cataract, abnormal iris transillumination, poor pupillary dilatation, persistent pupillary membranes, iridohyaloid adhesions, and prominent iris processes in the anterior chamber angle.